1. The document discusses the management of single ventricle physiology, which involves connecting the systemic and pulmonary circulations in parallel rather than series due to the inability to establish two independent functioning ventricles.
2. The management involves initial palliation through procedures such as the bidirectional Glenn shunt or pulmonary artery banding, followed by definitive palliation with a Fontan operation around 3 years of age to connect the systemic venous return directly to the pulmonary arteries without an interposing ventricle.
3. The Fontan operation has evolved over time from atrio-pulmonary connections to total cavopulmonary connections using intra-atrial tunnels or extracardiac conduits to more efficiently direct superior vena c
This document summarizes the management of single ventricle physiology from birth through surgery. It identifies how infants with this condition present, including shock, cyanosis, or heart failure. Key aspects of pre-operative stabilization include identifying obstructions to pulmonary or systemic blood flow and whether the atrial septum is restrictive. Surgical options like the Norwood procedure are described which aim to balance circulations prior to a Fontan-type completion. Post-operative care focuses on monitoring oxygen delivery and adjusting pulmonary and systemic resistances through ventilation or drugs.
- The document discusses the Fontan procedure for univentricular heart defects. It covers the evolution of the Fontan concept from the original atriopulmonary connection to lateral tunnel and extracardiac conduit techniques. It also discusses indications for Fontan, complications such as arrhythmias and ventricular dysfunction, and strategies to optimize outcomes like fenestration.
Single ventricle presentation for pediatricianLaxmi Ghimire
As the number of children who survive single ventricle physiology, it is very important for the pediatrician to understand about them to give them the best care.
Single ventricle refers to congenital heart defects where there is only one functional ventricle supporting both the pulmonary and systemic circulations. There are various classifications, and the goal of treatment is to balance blood flow between the lungs and body. Initial medical management uses prostaglandins and aims for balanced pulmonary flow. Later stages involve surgical procedures like shunts, banding of arteries, and ultimately the Fontan procedure to separate circulation to the lungs and body without overloading the single ventricle. Complications can include arrhythmias, heart failure, and protein-losing enteropathy. Long term outcomes are improved with careful patient selection and multi-stage management to optimize hemodynamics at each stage.
This document provides an overview of the Glenn shunt procedure for palliating certain congenital heart defects involving a single ventricle. It describes the history and development of cavopulmonary connections, the surgical techniques for performing bidirectional Glenn shunts, and the postoperative physiology and potential issues. A Glenn shunt involves connecting the superior vena cava directly to the pulmonary arteries, reducing the volume load on the single ventricle and allowing pulmonary blood flow without mixing with systemic venous return.
1. A ventricular septal defect (VSD) is an opening in the wall separating the ventricles that allows blood to shunt between them.
2. VSDs are the most common congenital heart defect in children and can be classified based on their location as membranous, perimembranous, muscular, inlet, or outlet.
3. A complete echocardiogram is needed to evaluate the location, size, direction of shunting, and effects of the defect. Three-dimensional echocardiography can help further define the anatomy and guide potential transcatheter closure of the VSD.
The document summarizes Fontan circulation, which is a surgical procedure that redirects blood flow from the inferior vena cava and superior vena cava directly to the pulmonary arteries, bypassing the right ventricle. It allows for systemic and pulmonary blood to circulate in parallel, driven by a single ventricle. The document discusses the history and evolution of the Fontan procedure, patient selection criteria, surgical techniques, postoperative physiology, and challenges.
This document summarizes the management of single ventricle physiology from birth through surgery. It identifies how infants with this condition present, including shock, cyanosis, or heart failure. Key aspects of pre-operative stabilization include identifying obstructions to pulmonary or systemic blood flow and whether the atrial septum is restrictive. Surgical options like the Norwood procedure are described which aim to balance circulations prior to a Fontan-type completion. Post-operative care focuses on monitoring oxygen delivery and adjusting pulmonary and systemic resistances through ventilation or drugs.
- The document discusses the Fontan procedure for univentricular heart defects. It covers the evolution of the Fontan concept from the original atriopulmonary connection to lateral tunnel and extracardiac conduit techniques. It also discusses indications for Fontan, complications such as arrhythmias and ventricular dysfunction, and strategies to optimize outcomes like fenestration.
Single ventricle presentation for pediatricianLaxmi Ghimire
As the number of children who survive single ventricle physiology, it is very important for the pediatrician to understand about them to give them the best care.
Single ventricle refers to congenital heart defects where there is only one functional ventricle supporting both the pulmonary and systemic circulations. There are various classifications, and the goal of treatment is to balance blood flow between the lungs and body. Initial medical management uses prostaglandins and aims for balanced pulmonary flow. Later stages involve surgical procedures like shunts, banding of arteries, and ultimately the Fontan procedure to separate circulation to the lungs and body without overloading the single ventricle. Complications can include arrhythmias, heart failure, and protein-losing enteropathy. Long term outcomes are improved with careful patient selection and multi-stage management to optimize hemodynamics at each stage.
This document provides an overview of the Glenn shunt procedure for palliating certain congenital heart defects involving a single ventricle. It describes the history and development of cavopulmonary connections, the surgical techniques for performing bidirectional Glenn shunts, and the postoperative physiology and potential issues. A Glenn shunt involves connecting the superior vena cava directly to the pulmonary arteries, reducing the volume load on the single ventricle and allowing pulmonary blood flow without mixing with systemic venous return.
1. A ventricular septal defect (VSD) is an opening in the wall separating the ventricles that allows blood to shunt between them.
2. VSDs are the most common congenital heart defect in children and can be classified based on their location as membranous, perimembranous, muscular, inlet, or outlet.
3. A complete echocardiogram is needed to evaluate the location, size, direction of shunting, and effects of the defect. Three-dimensional echocardiography can help further define the anatomy and guide potential transcatheter closure of the VSD.
The document summarizes Fontan circulation, which is a surgical procedure that redirects blood flow from the inferior vena cava and superior vena cava directly to the pulmonary arteries, bypassing the right ventricle. It allows for systemic and pulmonary blood to circulate in parallel, driven by a single ventricle. The document discusses the history and evolution of the Fontan procedure, patient selection criteria, surgical techniques, postoperative physiology, and challenges.
1) Atrial septal defects are one of the most common types of pre-tricuspid shunts and can often remain asymptomatic until later in life when they may lead to heart failure, pulmonary hypertension, or arrhythmias if left unrepaired.
2) The natural history and prognosis of atrial septal defects depends on factors like the size of the defect and age at diagnosis, with smaller defects having higher rates of spontaneous closure and repair at a younger age leading to better outcomes.
3) Device or surgical closure of atrial septal defects can successfully close the defect and improve symptoms, but the best outcomes are seen in those with less elevated pulmonary pressures and cardiac chamber enlargement prior to repair
The document discusses the single ventricle condition known as hypoplastic left heart syndrome (HLHS). It covers the embryology, anatomy, physiology, treatment options and stages of palliation including the Norwood procedure (Stage I), Glenn or Hemi-Fontan procedure (Stage II), and Fontan procedure (Stage III). The goal of staged surgical repairs is to gradually unload the single ventricle and optimize pulmonary and systemic blood flow to allow for growth prior to the final Fontan circulation.
1) Transthoracic and transesophageal echocardiography are important modalities for assessing atrial septal defects (ASDs). TTE can identify RV volume overload and septal flattening, while TEE precisely measures defect size and evaluates rim morphology.
2) The four main types of ASDs - ostium secundum, ostium primum, sinus venosus, and coronary sinus defects - have distinguishing echo features. Doppler can demonstrate shunt direction and magnitude.
3) Echocardiography guides percutaneous ASD closure by assessing defect and rim anatomy, device sizing, and post-procedure result. Understanding echo features is key to ensuring procedure success.
This document discusses the anatomy, embryology, and management of L-TGA (transposition of the great arteries). Some key points:
- In L-TGA, the ventricles are inverted such that the morphologic right ventricle is on the left and pumps blood to the lungs, while the morphologic left ventricle is on the right and pumps blood to the body.
- Embryologically, abnormal leftward looping of the heart tube during development results in the inverted ventricles. The conduction system and coronary arteries also have abnormal anatomy.
- Clinical features may include congenital heart block, progressive tricuspid regurgitation, pulmonary stenosis, and heart failure. Diagn
Single ventricle physiology refers to congenital heart defects where the entire atrioventricular junction connects to a single ventricular chamber. This includes conditions like double inlet left ventricle (DILV), tricuspid atresia, and unbalanced atrioventricular septal defects. The underlying embryology is not fully understood but is thought to involve limitations on inflow or outflow of the left ventricle. Initial management focuses on optimizing pulmonary and systemic blood flow without overloading the single ventricle. Surgical options have evolved from supportive care to staged reconstruction like the Norwood procedure and subsequent Fontan operations.
1) Cavopulmonary connections like the Glenn shunt divert systemic venous return directly to the pulmonary circulation, improving oxygen saturation for patients with single ventricle physiology.
2) The Glenn shunt involves anastomosis of the superior vena cava to the right pulmonary artery, reducing the volume load on the single ventricle.
3) Immediate postoperative issues include managing ventilation, elevated cavopulmonary pressures, hypertension/bradycardia, low cardiac output, and cyanosis which may result from pulmonary or systemic venous desaturation or decreased pulmonary blood flow.
Surgical management of tetralogy of Fallot has evolved significantly over time. Early repairs involved Blalock-Taussig shunts or direct anastomoses to increase pulmonary blood flow. Modern repairs typically involve closure of the ventricular septal defect through a ventriculotomy and placement of a transannular patch to address right ventricular outflow tract obstruction. Residual abnormalities are common after repair. Surgical techniques continue to be refined to minimize postoperative right ventricular dysfunction and pulmonary regurgitation. Assessment after repair and consideration of reintervention are important for long-term outcomes.
This document discusses various types of single ventricle heart defects where there is only one functioning ventricle pumping blood to both the lungs and body. It describes the different terms used to describe these hearts including single ventricle, univentricular heart, and double inlet ventricle. The most common type is double inlet left ventricle where both atria connect to a dominant left ventricle. Other types include double inlet right ventricle, absent atrioventricular connections, and a common atrioventricular valve. The document outlines the challenges these hearts face in maintaining adequate blood flow and oxygen levels to both circulations.
Single ventricle physiology involves a heart with only one functional pumping chamber. The document discusses the anatomy, physiology, and surgical management of various types of single ventricle hearts. Key points include: the goal of initial surgery is to provide unobstructed systemic outflow and pulmonary blood flow while limiting pulmonary pressures; manipulation of pulmonary and systemic vascular resistances is important for balancing blood flow; and inotropic support can increase cardiac output while adjusting pulmonary to systemic flow ratios.
1) Pulmonary regurgitation is a common consequence of tetralogy of Fallot repair and can lead to right ventricular dilation and dysfunction over time.
2) Echocardiography and cardiac MRI are useful for evaluating the severity of pulmonary regurgitation and assessing the degree of right ventricular dilation and dysfunction.
3) Indications for pulmonary valve replacement include moderate or severe pulmonary regurgitation with signs of right heart failure or dilation out of proportion to age. It aims to prevent irreversible right ventricular damage.
This document provides an overview of right heart catheterization (RHC) in children. It begins with a brief history of RHC, describing early experiments in the 1840s-1920s. The document then covers patient preparation, venous access approaches, conducting the procedure, normal pressure values, shunt detection/quantification using oximetry, and understanding Fick's principle. The key objectives are to gain knowledge on performing tailored RH studies, the diagnostic role of RHC, and quantifying left-to-right shunts.
This document discusses persistent truncus arteriosus, a congenital heart defect where a single arterial trunk arises from the heart to provide blood flow to the systemic, pulmonary, and coronary circulations. It describes the embryology, anatomy, classification, presentation, diagnosis, and treatment of the condition. Surgical repair aims to close the ventricular septal defect, commit the common trunk to the left ventricle, and reconstruct the right ventricular outflow tract. Outcomes have improved with early corrective surgery, though additional anomalies increase mortality risk.
Pulmonary artery banding (PAB) is a palliative surgical technique used to reduce pulmonary blood flow in infants with congenital heart defects. It involves placing a band around the pulmonary artery to create stenosis and decrease blood flow to the lungs. PAB is used as an initial intervention for defects causing pulmonary overcirculation to prevent congestive heart failure and pulmonary hypertension before a definitive repair. It is also used to prepare the left ventricle in some patients with transposition of the great arteries prior to later procedures. The goal of PAB is to reduce pulmonary pressures and improve systemic circulation. It remains an important technique for staged surgical treatment of certain congenital heart conditions.
1) Complete transposition of the great arteries (d-TGA) is a congenital heart defect where the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle, causing ventriculoarterial discordance.
2) In d-TGA, the systemic and pulmonary circulations are arranged in parallel rather than in series, requiring blood flow between the circuits through connections like an atrial or ventricular septal defect.
3) Echocardiography is useful for diagnosing d-TGA by demonstrating the aorta originating from the right ventricle and pulmonary artery from the left ventricle, as well as identifying the origins of the coronary arteries.
A 45 year old woman presented with shortness of breath on exertion. Echocardiography showed an atrial septal defect (ASD). ASDs are congenital heart defects where the wall separating the left and right atria is incomplete. The most common type is secundum ASD, which accounts for 70-75% of cases. ASDs allow blood to shunt from the left to the right atrium, overloading the right heart and lungs over time if not repaired. Echocardiography is the primary test to diagnose ASDs.
The document discusses the history and details of the arterial switch operation (ASO) for transposition of the great arteries (TGA). It provides a timeline of pioneers of techniques from 1959 to 1989. It then discusses specifics of the neonatal repair procedure, important technical aspects like coronary transfer techniques, and factors that influence long term outcomes like coronary patterns, ventricular function, and rhythm disturbances. It concludes with take home messages that earlier ASO leads to better results and the procedure now has a mortality rate of less than 5%, making it safe.
The univentricular repair indications, procedures, outcomes and controversiespatacsi
This document discusses surgical options for single ventricle heart defects, including the indications, timing, and outcomes of various palliative surgeries. It describes initial procedures like pulmonary artery banding or shunting to relieve obstruction and provide pulmonary blood flow. It then discusses staged repairs like the bidirectional Glenn procedure or hemi-Fontan that redirect blood flow. Finally, it covers the modified Fontan operation, which separates systemic and pulmonary circulation without a subpulmonary ventricle. Complications, long-term outcomes, and factors affecting success are also addressed.
This document discusses Eisenmenger syndrome, a condition where pulmonary hypertension develops due to increased blood flow through defects between the systemic and pulmonary circulations. It provides details on causes, clinical features, pathology findings, and treatments. Key points include:
- Eisenmenger syndrome is caused by defects like VSDs, ASDs, and PDA that allow high blood flow to the lungs and cause pulmonary hypertension over time.
- Common causes of death include hemoptysis from pulmonary artery ruptures, heart failure, and complications from attempted defect repair surgery.
- Pathological findings show thickened pulmonary arteries that resemble the fetal pattern and contribute to high pulmonary vascular resistance.
- Medical treatments are generally ineffective once int
Cardiopulmonary bypass (CPB) involves diverting blood from the heart to an external circuit for oxygenation and pumping. The basic components are a venous reservoir, oxygenator, heat exchanger, pump, and arterial filter. Initiation requires careful monitoring as the patient is transitioned to bypass. Management on CPB maintains appropriate pump flow, mean arterial pressure, temperature, and organ perfusion through monitoring of multiple parameters.
I am Dr Julieth Nachone Kabirigi from Mwanza, Tanzania United Republic
I am a Pediatric Cardiologist
Interested on sharing knowledge on Paediatric Cardiology subject.
1) Atrial septal defects are one of the most common types of pre-tricuspid shunts and can often remain asymptomatic until later in life when they may lead to heart failure, pulmonary hypertension, or arrhythmias if left unrepaired.
2) The natural history and prognosis of atrial septal defects depends on factors like the size of the defect and age at diagnosis, with smaller defects having higher rates of spontaneous closure and repair at a younger age leading to better outcomes.
3) Device or surgical closure of atrial septal defects can successfully close the defect and improve symptoms, but the best outcomes are seen in those with less elevated pulmonary pressures and cardiac chamber enlargement prior to repair
The document discusses the single ventricle condition known as hypoplastic left heart syndrome (HLHS). It covers the embryology, anatomy, physiology, treatment options and stages of palliation including the Norwood procedure (Stage I), Glenn or Hemi-Fontan procedure (Stage II), and Fontan procedure (Stage III). The goal of staged surgical repairs is to gradually unload the single ventricle and optimize pulmonary and systemic blood flow to allow for growth prior to the final Fontan circulation.
1) Transthoracic and transesophageal echocardiography are important modalities for assessing atrial septal defects (ASDs). TTE can identify RV volume overload and septal flattening, while TEE precisely measures defect size and evaluates rim morphology.
2) The four main types of ASDs - ostium secundum, ostium primum, sinus venosus, and coronary sinus defects - have distinguishing echo features. Doppler can demonstrate shunt direction and magnitude.
3) Echocardiography guides percutaneous ASD closure by assessing defect and rim anatomy, device sizing, and post-procedure result. Understanding echo features is key to ensuring procedure success.
This document discusses the anatomy, embryology, and management of L-TGA (transposition of the great arteries). Some key points:
- In L-TGA, the ventricles are inverted such that the morphologic right ventricle is on the left and pumps blood to the lungs, while the morphologic left ventricle is on the right and pumps blood to the body.
- Embryologically, abnormal leftward looping of the heart tube during development results in the inverted ventricles. The conduction system and coronary arteries also have abnormal anatomy.
- Clinical features may include congenital heart block, progressive tricuspid regurgitation, pulmonary stenosis, and heart failure. Diagn
Single ventricle physiology refers to congenital heart defects where the entire atrioventricular junction connects to a single ventricular chamber. This includes conditions like double inlet left ventricle (DILV), tricuspid atresia, and unbalanced atrioventricular septal defects. The underlying embryology is not fully understood but is thought to involve limitations on inflow or outflow of the left ventricle. Initial management focuses on optimizing pulmonary and systemic blood flow without overloading the single ventricle. Surgical options have evolved from supportive care to staged reconstruction like the Norwood procedure and subsequent Fontan operations.
1) Cavopulmonary connections like the Glenn shunt divert systemic venous return directly to the pulmonary circulation, improving oxygen saturation for patients with single ventricle physiology.
2) The Glenn shunt involves anastomosis of the superior vena cava to the right pulmonary artery, reducing the volume load on the single ventricle.
3) Immediate postoperative issues include managing ventilation, elevated cavopulmonary pressures, hypertension/bradycardia, low cardiac output, and cyanosis which may result from pulmonary or systemic venous desaturation or decreased pulmonary blood flow.
Surgical management of tetralogy of Fallot has evolved significantly over time. Early repairs involved Blalock-Taussig shunts or direct anastomoses to increase pulmonary blood flow. Modern repairs typically involve closure of the ventricular septal defect through a ventriculotomy and placement of a transannular patch to address right ventricular outflow tract obstruction. Residual abnormalities are common after repair. Surgical techniques continue to be refined to minimize postoperative right ventricular dysfunction and pulmonary regurgitation. Assessment after repair and consideration of reintervention are important for long-term outcomes.
This document discusses various types of single ventricle heart defects where there is only one functioning ventricle pumping blood to both the lungs and body. It describes the different terms used to describe these hearts including single ventricle, univentricular heart, and double inlet ventricle. The most common type is double inlet left ventricle where both atria connect to a dominant left ventricle. Other types include double inlet right ventricle, absent atrioventricular connections, and a common atrioventricular valve. The document outlines the challenges these hearts face in maintaining adequate blood flow and oxygen levels to both circulations.
Single ventricle physiology involves a heart with only one functional pumping chamber. The document discusses the anatomy, physiology, and surgical management of various types of single ventricle hearts. Key points include: the goal of initial surgery is to provide unobstructed systemic outflow and pulmonary blood flow while limiting pulmonary pressures; manipulation of pulmonary and systemic vascular resistances is important for balancing blood flow; and inotropic support can increase cardiac output while adjusting pulmonary to systemic flow ratios.
1) Pulmonary regurgitation is a common consequence of tetralogy of Fallot repair and can lead to right ventricular dilation and dysfunction over time.
2) Echocardiography and cardiac MRI are useful for evaluating the severity of pulmonary regurgitation and assessing the degree of right ventricular dilation and dysfunction.
3) Indications for pulmonary valve replacement include moderate or severe pulmonary regurgitation with signs of right heart failure or dilation out of proportion to age. It aims to prevent irreversible right ventricular damage.
This document provides an overview of right heart catheterization (RHC) in children. It begins with a brief history of RHC, describing early experiments in the 1840s-1920s. The document then covers patient preparation, venous access approaches, conducting the procedure, normal pressure values, shunt detection/quantification using oximetry, and understanding Fick's principle. The key objectives are to gain knowledge on performing tailored RH studies, the diagnostic role of RHC, and quantifying left-to-right shunts.
This document discusses persistent truncus arteriosus, a congenital heart defect where a single arterial trunk arises from the heart to provide blood flow to the systemic, pulmonary, and coronary circulations. It describes the embryology, anatomy, classification, presentation, diagnosis, and treatment of the condition. Surgical repair aims to close the ventricular septal defect, commit the common trunk to the left ventricle, and reconstruct the right ventricular outflow tract. Outcomes have improved with early corrective surgery, though additional anomalies increase mortality risk.
Pulmonary artery banding (PAB) is a palliative surgical technique used to reduce pulmonary blood flow in infants with congenital heart defects. It involves placing a band around the pulmonary artery to create stenosis and decrease blood flow to the lungs. PAB is used as an initial intervention for defects causing pulmonary overcirculation to prevent congestive heart failure and pulmonary hypertension before a definitive repair. It is also used to prepare the left ventricle in some patients with transposition of the great arteries prior to later procedures. The goal of PAB is to reduce pulmonary pressures and improve systemic circulation. It remains an important technique for staged surgical treatment of certain congenital heart conditions.
1) Complete transposition of the great arteries (d-TGA) is a congenital heart defect where the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle, causing ventriculoarterial discordance.
2) In d-TGA, the systemic and pulmonary circulations are arranged in parallel rather than in series, requiring blood flow between the circuits through connections like an atrial or ventricular septal defect.
3) Echocardiography is useful for diagnosing d-TGA by demonstrating the aorta originating from the right ventricle and pulmonary artery from the left ventricle, as well as identifying the origins of the coronary arteries.
A 45 year old woman presented with shortness of breath on exertion. Echocardiography showed an atrial septal defect (ASD). ASDs are congenital heart defects where the wall separating the left and right atria is incomplete. The most common type is secundum ASD, which accounts for 70-75% of cases. ASDs allow blood to shunt from the left to the right atrium, overloading the right heart and lungs over time if not repaired. Echocardiography is the primary test to diagnose ASDs.
The document discusses the history and details of the arterial switch operation (ASO) for transposition of the great arteries (TGA). It provides a timeline of pioneers of techniques from 1959 to 1989. It then discusses specifics of the neonatal repair procedure, important technical aspects like coronary transfer techniques, and factors that influence long term outcomes like coronary patterns, ventricular function, and rhythm disturbances. It concludes with take home messages that earlier ASO leads to better results and the procedure now has a mortality rate of less than 5%, making it safe.
The univentricular repair indications, procedures, outcomes and controversiespatacsi
This document discusses surgical options for single ventricle heart defects, including the indications, timing, and outcomes of various palliative surgeries. It describes initial procedures like pulmonary artery banding or shunting to relieve obstruction and provide pulmonary blood flow. It then discusses staged repairs like the bidirectional Glenn procedure or hemi-Fontan that redirect blood flow. Finally, it covers the modified Fontan operation, which separates systemic and pulmonary circulation without a subpulmonary ventricle. Complications, long-term outcomes, and factors affecting success are also addressed.
This document discusses Eisenmenger syndrome, a condition where pulmonary hypertension develops due to increased blood flow through defects between the systemic and pulmonary circulations. It provides details on causes, clinical features, pathology findings, and treatments. Key points include:
- Eisenmenger syndrome is caused by defects like VSDs, ASDs, and PDA that allow high blood flow to the lungs and cause pulmonary hypertension over time.
- Common causes of death include hemoptysis from pulmonary artery ruptures, heart failure, and complications from attempted defect repair surgery.
- Pathological findings show thickened pulmonary arteries that resemble the fetal pattern and contribute to high pulmonary vascular resistance.
- Medical treatments are generally ineffective once int
Cardiopulmonary bypass (CPB) involves diverting blood from the heart to an external circuit for oxygenation and pumping. The basic components are a venous reservoir, oxygenator, heat exchanger, pump, and arterial filter. Initiation requires careful monitoring as the patient is transitioned to bypass. Management on CPB maintains appropriate pump flow, mean arterial pressure, temperature, and organ perfusion through monitoring of multiple parameters.
I am Dr Julieth Nachone Kabirigi from Mwanza, Tanzania United Republic
I am a Pediatric Cardiologist
Interested on sharing knowledge on Paediatric Cardiology subject.
Swan-Ganz catheters are balloon-tipped catheters inserted into the heart to measure pressures and collect blood samples from the right atrium, right ventricle, and pulmonary artery. This allows clinicians to assess conditions like shock, respiratory distress, and complications of myocardial infarction. Measurements of pressures, oxygen saturations, and cardiac output can guide therapy for critical illnesses and help evaluate the effects of treatments. While useful for management, the procedure does carry risks of complications if not performed carefully.
The Fontan procedure is a palliative surgery for patients born with certain congenital heart defects involving a single functional ventricle. It involves redirecting systemic venous blood directly to the lungs, without passing through a ventricle. While it provides improved survival and quality of life, long term complications can develop due to the unnatural circulation. Common complications include arrhythmias, protein losing enteropathy, liver disease, and pulmonary issues. Regular screening is important to monitor for these complications.
1. The document discusses cardiovascular (CVS) monitoring in critical care, including the purposes, effectiveness, and common variables monitored such as heart rate, blood pressure, oxygen saturation, and more.
2. It describes the methods of monitoring various CVS variables, both invasively like arterial and pulmonary artery catheters, and non-invasively like pulse oximetry. Potential complications of different monitoring methods are also outlined.
3. The document provides details on interpreting CVS monitoring parameters and emphasizes the importance of considering the clinical context and pathophysiology of the patient's condition when evaluating monitoring data.
This document provides an overview of cardiac monitoring techniques including pulmonary artery catheters, transesophageal echocardiography, and coagulation monitoring. It discusses the indications, contraindications, and complications of pulmonary artery catheters. It also describes the waveforms seen in different cardiac chambers and how they can help assess cardiac function. Transesophageal echocardiography views and the structures they allow viewing are outlined. Coagulation monitoring with heparin and the activated clotting time during cardiac surgery is summarized, along with complications like heparin resistance and heparin-induced thrombocytopenia.
management of portal hypertension by Dr.ZarinWaqas Khalil
Portal hypertension is increased blood pressure in the portal vein system that connects the digestive organs and liver. This document summarizes the anatomy, causes, symptoms, and management of portal hypertension. It describes diagnostic techniques like endoscopy and treatments including pharmacotherapy, transjugular intrahepatic portosystemic shunting (TIPS), surgical shunting procedures, and liver transplantation. The management of acute variceal bleeding involves initial endoscopic therapy followed by additional interventions or shunt procedures to prevent rebleeding.
The document discusses pulmonary artery catheter monitoring and measurements. It provides details on:
- The history and uses of pulmonary artery catheters
- Physiological measurements that can be obtained from pulmonary artery catheters including pressures, oxygen saturations, and derived variables
- Placement technique for pulmonary artery catheters and ensuring accurate measurements
- Interpretation of catheter waveforms and pressures from different locations within the heart and how they are affected in different disease states
1 Monitoring of Central Venous Pressure & Its Techniquessrinivas8990
This document discusses monitoring central venous pressure through central venous catheters. It describes the types of central lines, their indications and contraindications, and techniques for insertion. Factors that affect central venous pressure are outlined, as are methods to measure pressure directly through transducers or indirectly by inspecting jugular veins. The document interprets central venous pressure waveforms and describes how pressure changes with respiration.
Weaning from cardiopulmonary bypass (CPB) requires optimizing several cardiovascular parameters to ensure a smooth transition back to native heart function. Key factors to address include adequate rewarming, hemodynamic stability, cardiac contractility, oxygen delivery, electrolyte/acid-base balance, and removal of air from the heart. The sequence of events involves gradually reducing pump flow while monitoring pressures and cardiac function. Inotropic support may be needed for patients at high risk of low output. Complications can include low cardiac output, arrhythmias, hypotension or hypertension, and end-organ dysfunction if not managed appropriately.
This document discusses central venous pressure (CVP), including its indications, measurement sites, determinants, and limitations. Some key points:
- CVP is the pressure measured in central veins close to the heart and reflects right atrial pressure. It provides information about right ventricular preload but does not indicate blood volume.
- CVP can be measured through the internal jugular, femoral, or subclavian veins. Factors like cardiac function, vascular compliance, blood volume, and intrathoracic pressure determine CVP.
- While CVP provides data on circulatory equilibrium between the heart and veins, it does not predict fluid responsiveness or tissue perfusion. Dynamic variables obtained through fluid challenges or
The Norwood procedure is the first of three surgeries required to treat single-ventricle conditions such as hypoplastic left heart syndrome (HLHS). Because the left side of the heart can’t be fixed, the series of surgeries rebuilds other parts of the heart.
The Norwood procedure is performed in the baby’s first or second week of life.to redirect the blood flow.
Three goals for the Norwood procedure:
1, Build a new aorta.
2, Direct blood from the right ventricle through the new aorta and on to the rest of the body.
3, Direct the right ventricle to pump blood to the lungs until the next surgery.
The major physiological effects of counterpulsation include:
A) increased coronary artery perfusion, increased preload, decreased after load, decreased myocardial oxygen consumption
Cardiac surgery is more difficult than other types of surgery due to the moving heart organ containing blood which is vital with no room for mistakes. Historical milestones like the heart-lung machine in 1937 and first coronary artery bypass graft in 1958 allowed cardiac surgery to become viable. Indications for cardiac surgery include CABG, valve repair/replacement, arrhythmia management, and congenital heart defects. Preop preparation assesses patient risk factors. During surgery, a heart-lung machine is used to bypass the heart and oxygenate blood while the surgeon operates. Common procedures like CABG graft arteries to improve blood flow. Postop care focuses on complications like hypothermia, bleeding, and low blood pressure.
1. Clinical examination alone is not sufficient to assess hemodynamic status in critically ill patients as individual vital signs do not reflect overall status.
2. Arterial lines can be used to monitor blood pressure, heart rate, and derive parameters like cardiac output but waveforms require interpretation and may be affected by various artifacts.
3. Pulmonary artery catheters can measure central venous and pulmonary artery pressures as well as cardiac output but have potential complications and their use remains controversial with no proven benefits shown in large trials.
1. Large v-waves seen on the monitor during PAC placement suggest increased left atrial pressure.
2. A patient experiencing symptoms during catheterization like tachypnea and hypotension likely has a punctured vessel, and left lateral positioning could help diagnose and treat a potential pneumothorax.
3. Inadvertently injecting a small amount of air into a PAC is unlikely to cause serious harm and may only result in transient dyspnea.
Intra Aortic Balloon Pump (IABP) 2009.ppttaimourali64
The document provides information about intra-aortic balloon pumps (IABP), including what they are, their purpose, insertion techniques, positioning, triggering, waveforms, complications, and weaning. An IABP is a volume displacement device that inflates and deflates in the aorta to reduce workload on the heart. It is used for hemodynamic support in conditions like heart failure and support during surgery. Proper positioning and triggering are important for the IABP to function effectively and safely.
1. The document discusses the history and techniques of intracranial pressure (ICP) monitoring. It describes historical figures who contributed to the understanding of ICP and various monitoring methods that have been developed over time.
2. The current gold standard for ICP monitoring is an external ventricular drain, though fiberoptic and strain gauge monitors provide alternatives. Newer methods like optic nerve sheath ultrasound provide noninvasive options.
3. Careful analysis of ICP waveforms can provide insights into intracranial compliance and dynamics that help guide management of conditions with elevated ICP like traumatic brain injury.
surgical approach of cyanotic congenital heart diseasedibufolio
This document discusses the surgical approaches for cyanotic congenital heart disease. It begins with an introduction and overview of topics to be covered, including fetal and adult circulation physiology. It then discusses various defects such as transposition of the great arteries, tetralogy of Fallot, and total anomalous pulmonary venous return. It outlines the history of various palliative and corrective surgeries performed over time, such as Blalock-Taussig shunts, cavopulmonary connections, and arterial switch operations. It provides guidance on timing of surgeries based on disease type and clinical status. It also discusses complications of the Fontan procedure. The document aims to close gaps in understanding regarding why certain surgeries are
Syncope refers to a sudden, transient loss of consciousness due to decreased blood flow to the brain. In children and adolescents, cardiac abnormalities causing syncope are rare, and the most common causes are autonomic syncope, vasovagal syncope, and postural hypotension. Evaluation of syncope in children aims to determine if there is an increased risk of sudden cardiac death. Factors suggesting a cardiac cause include lack of prodrome, palpitations, exercise-induced syncope, family history of cardiac issues or early death, and prior known cardiac disease. These patients should be referred to a cardiologist to evaluate for potential inherited cardiac conditions like Brugada syndrome or long QT syndrome.
The document outlines a talk on fetal cardiac screening. It discusses the impact of screening from both a health and parental perspective. Screening allows for planning delivery at centers equipped for any needed care. It can identify life-threatening conditions for in utero treatment or help avoid unnecessary post-birth testing and transport. Parents benefit from informed decisions and preparation. The talk explores current practices and potential future directions for improving screening.
This document discusses syncope in children and adolescents, providing definitions of syncope and sudden cardiac death. It notes that cardiac abnormalities are a rare cause of syncope in children unlike in adults. Various types of autonomic syncope are described including vasovagal, postural hypotension, and dyautonomia. Evaluation for cardiac syncope is recommended to determine risk of death. Specific cardiac conditions like Brugada syndrome, long QT syndrome, short QT syndrome, and catecholaminergic polymorphic ventricular tachycardia are discussed. The take home message is to consider arrhythmias with any exertional or unexplained syncope and to evaluate family history of sudden cardiac death or arrhythmias.
This document discusses two case scenarios involving pediatric cardiology patients. The first scenario is about a 12-year-old boy who is overweight due to a sedentary lifestyle involving online schooling, TV watching, and eating fast and junk food. The second scenario describes a 14-year-old active boy whose grandfather died at age 49 and who has elevated cholesterol and LDL levels. The document discusses the long-term cardiovascular risks of obesity and high cholesterol in childhood, including increased blood pressure, lipids, and future risk of atherosclerosis. It recommends addressing these risks through lifestyle changes, physical activity, healthy eating, and early screening and treatment when needed, including behavioral interventions and statin prescriptions in rare cases of familial hyper
This document discusses the percutaneous closure of a residual aortopulmonary window in a 2-year-old boy using a second occlusion device. The boy was originally treated for an aortopulmonary window at 2 months of age, but follow up showed a residual 4mm window. A second device, an ADO-II 6x6, was successfully deployed above the original device with no residual shunt seen. The boy was discharged the next day with both devices in position and no complications. The summary demonstrates the feasibility of using a second occlusion device to treat residual defects after an initial device closure procedure.
Long term Post operative care of a child with CHD involves monitoring for potential issues like reintervention due to lesion recurrence, arrhythmias, and thromboembolism. It also requires assessing schooling, neurodevelopment, exercise capacity, and providing guidance on marriage, pregnancy, and contraception. Children with repaired lesions are most at risk for reintervention and arrhythmias compared to those with completely corrected or palliated lesions. While outcomes have improved, lifelong multidisciplinary care is generally needed due to the risk of ongoing medical, developmental, and social issues.
This document discusses the timing of interventions for congenital heart defects (CHDs) in pediatric patients. It involves balancing factors like hemodynamic severity, natural history of the defect, procedural outcomes, and institutional experience. There is a trend toward early correction which avoids adverse consequences and is now feasible and realistic in Indian centers with excellent results. The document then discusses scenarios involving ventricular septal defects, atrial septal defects, and patent ductus arteriosus, when intervention is recommended based on symptoms, size of defect, and age/weight of the patient. It notes most CHDs can now be corrected with long-term survival and without need for reintervention in 85-90% of cases.
This document provides an overview of various echocardiographic views used to visualize cardiac structures and diagnose pathologies. It describes the apical and parasternal views including the 4-chamber, 5-chamber, 2-chamber, and short-axis views. Each view is explained in terms of the structures seen and pathologies that can be identified. Measurement techniques like Simpson's method for ejection fraction are also summarized.
This document discusses the timing of interventions for various congenital heart defects (CHDs) in pediatric patients. It provides several case scenarios to illustrate the decision-making process. The key factors in determining when to intervene include the hemodynamic severity of the defect, its predicted natural history, procedural outcomes, and institutional experience. Early correction is generally preferred to avoid complications, and is now feasible in selected centers with excellent results. The document reviews timing guidelines for specific defects such as ventricular septal defects and patent ductus arteriosus. It also describes treatment options such as surgical closure or transcatheter device closure for different CHDs.
A 12-year-old child is overweight with a BMI of 31 kg/m2 due to a sedentary lifestyle. Obesity increases the risk of high blood pressure, diabetes, and cardiovascular disease over the long term. Management involves promoting outdoor physical activity and healthy eating habits.
A 14-year-old boy has a family history of early death from cardiovascular disease. Though active, he has elevated cholesterol levels placing him at risk for future cardiovascular problems. Early detection and lifestyle modifications can help prevent disease progression.
Screening children's cholesterol levels starting at age 10 and 19 allows for early identification of risk and targeted prevention and treatment approaches like lifestyle changes and low-dose statin therapy for those at highest risk
Micronutrient deficiency is common among children globally. At least 50% of children aged 6 months to 5 years suffer from one or more micronutrient deficiencies such as iron, iodine, folate, and vitamin A. The reasons for the high prevalence include inadequate intake of nutritious foods, poor absorption of minerals and vitamins, frequent infections, and consumption of refined foods. Micronutrients play an important role in brain development during the first 1,000 days of life. Complementary feeding starting at 6 months is important to meet increased nutrient needs but current practices in India are often inadequate. Ensuring adequate nutrition through home-made preparations, breastfeeding, and fortified complementary foods can help bridge nutrition
1. Caesarean section delivery disrupts the normal pattern of microbial colonization in infants, dominating their gut with bacteria from the human skin and oral cavity rather than vaginal and enteric bacteria acquired during a vaginal birth.
2. Microbial dysbiosis resulting from C-section delivery has been associated with increased risk of conditions like asthma, obesity, food allergies, and inflammatory bowel disease later in life.
3. Restoring a healthy gut microbiota in infants born by C-section through breastfeeding and probiotic supplementation may help reduce the risk of these long-term health issues, though more research is still needed.
This document discusses the current state of pediatric cardiac services in India. It notes that India has a high birth prevalence of congenital heart disease (CHD), with approximately 242,390 children born with CHD each year. However, the availability of advanced cardiac care is very limited, with only 9 high-volume centers performing over 500 surgeries per year. While an estimated 43,000 children are born annually with serious forms of CHD requiring treatment, only around 8,500 (20%) currently receive optimal cardiac care. The document outlines several challenges facing the improvement and expansion of pediatric cardiac services in India, including limited resources, infrastructure, and trained staff. It proposes various strategies to address these issues, such as establishing more specialized
1. Heart diseases in children, specifically congenital heart diseases (CHDs), are not as rare as once thought, occurring in 8-10 out of every 1,000 live births and representing the most common birth defect.
2. CHDs can present in a variety of ways from cyanosis and congestive heart failure in neonates to symptoms appearing later in childhood. Determining if a CHD is present, whether it involves cyanosis, and the specific malformation involved requires consideration of various clinical criteria and tests.
3. Proper diagnosis and management is important as some CHDs depend on persistent ductal circulation after birth for survival, requiring treatment with prostaglandins to keep the ductus arteriosus
1. A 65-year-old woman presented with a fall and hip fracture. Her ECG showed complete heart block with a ventricular rate of 45 beats per minute, requiring a permanent pacemaker.
2. A 50-year-old woman with rheumatic heart disease was in atrial fibrillation with a controlled ventricular rate of 60-65 beats per minute on her ECG. The ECG showed signs of digoxin use and possible hypokalemia, requiring questions about appetite and potassium levels.
3. A 20-year-old woman presented with chest pain and her ECG showed a pattern consistent with Wolff-Parkinson-White syndrome type B with right posterior accessory pathway location
This document provides an overview of congenital heart diseases (CHDs):
1. CHDs are defects in heart structure and function that exist at birth, though symptoms may not appear until childhood or later. They occur in approximately 1% of live births and are a leading cause of birth defect mortality.
2. CHDs can be acyanotic (without cyanosis) or cyanotic (with cyanosis) and include defects such as ventricular septal defects (VSD), atrial septal defects (ASD), patent ductus arteriosus (PDA), tetralogy of Fallot, and transposition of the great arteries.
3. While the exact causes of most CHDs are unknown
This document provides an overview of examining the cardiovascular system in pediatric patients and approaching a child with suspected congenital heart disease (CHD). It discusses taking a history, performing a general physical exam, assessing vital signs, inspecting the precordium, palpating pulses and heart sounds, and auscultating for murmurs. The key steps in approaching a child with possible CHD are determining if it is cyanotic or acyanotic, assessing pulmonary blood flow and pulmonary artery pressure, and identifying any duct-dependent lesions.
A 2 day old term neonate presented with tachypnea and a heart rate of 90%, raising suspicion of a congenital heart disease (CHD). To determine if the neonate had a CHD, the doctor considered performing a hyperoxia test, applying Nada's criteria, and conducting an echocardiogram. If a CHD was confirmed, the next steps would be to determine if the child had cyanotic or acyanotic heart disease, increased or normal pulmonary blood flow, pulmonary arterial hypertension, and duct dependency to guide referral to a pediatric cardiologist.
- The document summarizes a cardiac catheterization report for a 16-month-old female patient with a history of cyanosis, murmur, and failure to gain weight appropriately who was diagnosed with congenital heart disease.
- Examination findings included cyanosis, low oxygen saturation, murmur, and signs of right ventricular hypertrophy. Previous echocardiogram showed dextro-transposition of the great arteries, ventricular septal defect, and pulmonary stenosis.
- The cardiac catheterization measured pressures in the heart chambers and major vessels. It found elevated pulmonary artery and right-sided heart pressures consistent with pulmonary hypertension. Calculations from the data determined abnormal pulmonary and systemic vascular resistances and a low ratio of
- The patient is a 3 year old female with a history of lung infections as an infant and diagnosis of congenital heart disease requiring surgery at age 1 year.
- Examination found signs of right heart enlargement and a loud heart murmur. Echocardiogram showed a large ventricular septal defect with left-to-right shunting and mild pulmonary artery hypertension.
- Cardiac catheterization was performed and found severe pulmonary artery hypertension with right ventricular and pulmonary artery pressures elevated. Oxygen saturations were low in the veins and pulmonary artery.
More from Dr. Murtaza Kamal MD,DNB,DrNB Ped Cardiology (20)
share - Lions, tigers, AI and health misinformation, oh my!.pptxTina Purnat
• Pitfalls and pivots needed to use AI effectively in public health
• Evidence-based strategies to address health misinformation effectively
• Building trust with communities online and offline
• Equipping health professionals to address questions, concerns and health misinformation
• Assessing risk and mitigating harm from adverse health narratives in communities, health workforce and health system
- Video recording of this lecture in English language: https://youtu.be/kqbnxVAZs-0
- Video recording of this lecture in Arabic language: https://youtu.be/SINlygW1Mpc
- Link to download the book free: https://nephrotube.blogspot.com/p/nephrotube-nephrology-books.html
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Overall life span (LS) was 1671.7±1721.6 days and cumulative 5YS reached 62.4%, 10 years – 50.4%, 20 years – 44.6%. 94 LCP lived more than 5 years without cancer (LS=2958.6±1723.6 days), 22 – more than 10 years (LS=5571±1841.8 days). 67 LCP died because of LC (LS=471.9±344 days). AT significantly improved 5YS (68% vs. 53.7%) (P=0.028 by log-rank test). Cox modeling displayed that 5YS of LCP significantly depended on: N0-N12, T3-4, blood cell circuit, cell ratio factors (ratio between cancer cells-CC and blood cells subpopulations), LC cell dynamics, recalcification time, heparin tolerance, prothrombin index, protein, AT, procedure type (P=0.000-0.031). Neural networks, genetic algorithm selection and bootstrap simulation revealed relationships between 5YS and N0-12 (rank=1), thrombocytes/CC (rank=2), segmented neutrophils/CC (3), eosinophils/CC (4), erythrocytes/CC (5), healthy cells/CC (6), lymphocytes/CC (7), stick neutrophils/CC (8), leucocytes/CC (9), monocytes/CC (10). Correct prediction of 5YS was 100% by neural networks computing (error=0.000; area under ROC curve=1.0).
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4. CONTENT OF THE TALK…
● Introduction
● SV management concept
● Indications
● Evolution of SV management
● Fontan physiology
● Sequelae of Fontan operation
● Treatment of circulatory failure
● Complications of Fontan operation
● Post-op follow up
● Long term results
4
5. INTRODUCTION
● Normal post natal CV consists:
● Double circuits connected in series
—Systemic
—Pulmonary
● Powered by double pump: Rt+ lt heart
5
6. SINGLE VENTRICLE…
● Systemic+ pulmonary circulation: Not connected
in series but in parallel
● Major disadvantages:
◦ Arterial desaturation
◦ Chronic volume overload to SV—> In due course
impairs ventricular function
6
7. Surgical definition…
● Inability to establish 2 independent
functioning ventricles to support in series
● Inadequate ventricular size+ volume
● Lack of independent inflow
7
9. General approach…
● Maintain adequate systemic perfusion+ o2
sats
● Maintain normal ventricular function
● Prevent ventricular HT/ dilatation
● Establish systemic venous to PA
connection
● Prevent/ control arrhythmias
9
10. INTRO CONT…
● Effects of chronic volume overload on SV :
1. Dilatation of atrium and ventricle
2. Eccentric hypertrophy
3. Spherical remodelling with reorientation of
wall fibers
4. Annular dilatation causing progressive AV
valve regurgitation
10
11. INTRO CONT…
● Hemodynamic problems in univentricular heart
arises from :
1. Lack of inter-ventricular coupling
2. Volume overload on SV
3. Mechanics of morphologic RV vs. LV
4. Morphology and functional state of AV valves
5. Degree of mixing within SV
6. PVR
7. Presence of pulmonic or subaortic stenosis
11
12. Indications of early intervention…
● Systemic outflow obstruction
● Severe cyanosis
● Increased PBF
● Obstruction to pulmonary venous return
12
16. Pre BDG ECHO evaluation…
● PA anatomy
● Assessment of PBF
● No of SVCs
● IVC interruption
● AVVR
● Size of IA communication
16
17. Additional surgical measures in
Glenn…
● Ligation of azygous
● PA plasty
● Interruption of forward flow
● Repair of AV valve
● Repair of systemic outflow obstruction
17
18. Visualisation of BDG shunt…
● Suprasternal short axis view
● Use low velocity scale: Venous flow
● PD shows biphasic low velocity forward
flow signal
18
19. Post BDG evaluation…
● Flow across Glenn
● PA distortion
● Decompressing veins
● Pulmonary AV malformation
19
20. The hemifontan procedure…
• Anastomosis between SVC-RA confluence+
central and BPAs to direct SVC blood to
pulmonary circulation
• Homograft patch augmentation of central+ BPAs
• Interruption of SVC blood from reaching RA using
homograft dam
• Interruption of antegrade PBF by transecting
MPA+ closing its cardiac end and ligating all other
sources of PBF
• Additional procedures as atrial septectomy, repair
of TAPVC, aortic valve repair, etc., in preparation
for future Fontan operation
20
22. Advantages of hemi fontan…
● Better caval offset
● Ease of future lateral tunnel Fontan
operation
● Augmentation of PAs
● Protection of phrenic nerve
● Superior hemodynamics
22
23. Limitations of hemi fontan…
• Technically more difficult: Use of a pulmonary
homograft, augmentation of central+ BPAs,
anastomosis of SVC-RA confluence toPAs needs
expertise with a definite learning curve
• Future extracardiac Fontan is difficult option since
PAs already bear an anastomosis with RA
• Suture line through SVC-RA confluence can
damage SA nodal artery leading to arrhythmias
later if one is not careful at initial operation
• No large scale prospective data is available to
study results of procedure in detail
23
26. Fontan Circulation
● Systemic venous return enters pulmonary
circulation: Without an interposing ventricle
● Shunts at venous, atrial, ventricular and arterial
level interrupted
● Places systemic and pulmonary circulations in
series driven by SV
26
27. Fontan cont…
● Advantages of a Fontan circuit :
◦ (Near) Normalisation of arterial saturation
◦ Abolishment of chronic volume overload on SV
● Cost for such circulation :
◦ Chronic systemic venous hypertension and congestion
◦ Decreased CO: Both at rest+ exercise
● CO: NO LONGER DETERMINED BY HEART, BUT BY
TRANSPULMONARY FLOW (IN TURN REGULATED
BY PVR)
27
28. Paradox of Fontan Circulation
● Normally: Good hemodynamic state has low
pressure in IVC(< 10 mmHg) and mPAP atleast 15
mmHg
● In Fontan: Systemic venous hypertension and
pulmonary arterial hypotension
● This pressure gradient: Driving force for PBF,
assisted mechanically by thoracic muscles+
respiratory function
28
29. INDICATIONS
● Cardiac malformation and a single functional ventricle
resulting from:
◦ Dysfunctional heart valve
◦ Absent/ inadequate pumping chamber
● MC CHDs palliated with Fontan circulation:
◦ TA
◦ PA-IVS
◦ HLHS
◦ DILV
29
30. Ideal Patient For Fontan :1977, Choussat et al
1. Age > 4 and < 15 years
2. Normal sinus rhythm
3. Normal SV connections
4. Normal RA volume
5. Mean PA pressure ≤ 15 mmHg
6. PVR < 4 Woods units/m2
7. Adequate-sized PA (PA to aortic diameter ratio ≥ 0.75)
8. LVEF ≥ 60%
9. Competent mitral valve (absence of mitral valve
insufficiency)
10. Absence of PA distortion
30
31. Commandements condensed…
● With increasing experience: More flexible
● 2 most important commandments in present era:
◦ Pre-op impaired ventricular function and
◦ Elevated PA pressures
31
32. Cardiac Catheterization For Pre-fontan
Evaluation :
● Patency of glenn shunt with analysis of PA
anatomy :
◦ Good sized PA without distortion
◦ Confluence stenosis (stenosis > 50% as compared to
adjacent segment)
◦ Presence or absence of anomalously draining
pulmonary veins in levophase
◦ Any significant decompressing venovenous collaterals
32
33. Cardiac Catheterization For Pre-fontan
Evaluation :
● IVC angiogram :
◦ Rule out interrupted IVC
◦ Rule out stenosis/ duplication of IVC
◦ Drainage pattern of hepatic veins
● Ventriculogram :
◦ Ventricular contractility
◦ AVVR
◦ Systemic outflow obstruction
◦ Antegrade flow from ventricle to PA
33
34. Cardiac Catheterization For Pre-fontan
Evaluation :
● Hemodynamic evaluation :
◦ PA pressures (systolic/diastolic/mean)
◦ PVR
◦ LVEDP
● 2 most important cath data (both mandatory)
◦ PA pressure
◦ PVR
34
35. Is cardiac catheterization mandatory?
● NO
● Alternative: Cardiac MRI
● Consensus opinion: Pre-op cath study must
◦ Only valid method to measure PVR
◦ Abnormal AP/ VV collaterals identified: Can be
embolized at same instance
35
36. EVOLUTION OF FONTAN OPERATION
● Since its original description: Numerous
modifications
● Helped advance the procedure+improved patient
outcomes
● Early 1980s, NORWOOD:“Novel Concept” : Using
RV to serve systemic circulation
● This discovery created a large new population of
candidates for Fontan palliation
36
38. Original Fontan Procedure
38
• SVC to RPA anastomosis
(Unidirectional Glenn shunt)
• Anastomosis of RA appendage to
LPA directing IVC flow through a
valved homograft
•Closure of ASD
39. Problems :
● RA dilated and lost contractile function
▪ Turbulence and energy loss
▪ Decreased pulmonary blood flow
▪ Stasis and thrombosis
● RA–pulmonary circuits: Obsolete
39
40. Total Cavo-Pulmonary Connection
● 1987: de Leval et al (Lateral tunnel)
◦ Direct connection between each vena cava and PA
◦ Bypass the right atrium and right ventricle
● In 1990, Marcellati (Extra cardiac fontan)
◦ Extra cardiac interposition graft b/w IVC and PA
● Advantages :
◦ More efficient cavopulmonary blood flow
◦ Reduce risk for arrhythmia and thrombosis
40
41. PRESENT DAY…
● Connecting SVC+ IVC to RPA
● Originally performed at same time
● Resulted in a marked increase in blood flow to
lungs: Pulmonary lymphatic congestion+ pleural
effusions
● No longer performed together
41
42. TCPC:
● Currently done in 2 stages :
● To allow body to adapt to different hemodynamic
states
● Reduce overall surgical morbidity and mortality
● Allows a better patient selection and intermediate
preparatory interventions
42
43. TCPC Cont…
● No ventricle to pump blood through lungs:
Elevated PAH—> Absolute contraindication
● At birth, impossible to create a Fontan
circulation:
◦ PVR is still raised for several weeks
◦ Caval veins and pulmonary arteries: Too small
43
44. Present Day Fontan
● Initially in the neonatal period, management must
aim to achieve :
1. Unrestricted flow from the heart to the aorta
● Coarctectomy
● Norwood repair
2. Well balanced limited flow to the lungs
● PA Banding
● Modified BTT shunt
3. Unrestricted return of blood to the ventricle
● Rashkind balloon septostomy
44
45. BDG / Hemi-fontan
● At 4–12 months of age
● First half of creating a total cavopulmonary circulation
circuit
● End-to-side anastomosis between SVC & RPA
● RPA is not divided, resulting in blood flow from SVC
into RPA+LPA
● Children may remain cyanotic because blood from the
IVC is not directed to lungs
45
46. Completion of Fontan
● 3–5 years of age
● IVC connected to PA with a conduit
● SVC flow is already directed into RPA by a
previous BDG
46
47. TCPC…
● Can be done in 2 methods:
◦ Internal conduit: Pass through RA chamber
◦ External conduit: Run completely outside heart to right
side of RA
47
48. Intra-atrial Tunnel Method
48
•Conduit is constructed with both the
lateral wall of RA and prosthetic material
•Inferior aspect of tunnel: Anastomosed
to IVC
• Superior aspect: Anastomosed to PA
49. ADVANTAGES…
● Conduit enlarges as child grows: May be used in
children as young as 1 year old
◦ Decreased blood stasis and risk of thrombosis
◦ Limited portion of RA exposed to high venous
pressures (reduces risk of arrhythmias)
◦ Coronary sinus remains in low pressure atrium (allows
unimpended myocardial venous drainage)
49
50. Extracardiac Conduit Method
● Usually performed only in
pts. > 3 years
● PTFE tube graft is placed
between transected IVC+PA
bypassing RA
50
51. Extra cardiac conduit…
● Advantages :
◦ No/ minimal CPB
◦ Entire atrium is left with low pressure: Less atrial distention,
arrhythmia and thrombosis
◦ Avoids RA incisions and extensive atrial sutures
◦ Reduces risk of sinus node injury
◦ Reduces incidence of post op arrhythmias
● Disadvantages :
◦ Cannot enlarge as child grows
◦ Performed only in child large enough to accept a graft of adequate size
to allow adult IVC blood flow
◦ Risk of obstruction by thrombus formation or neo intimal hyperplasia
51
53. Fenestrated Fontan
● Small opening or fenestration may be created between
conduit and RA
● Functions as a pop-off valve (Rt—> Lt shunt)
◦ Prevents rapid volume overload to lungs
◦ Limit caval pressure
◦ Increase preload to systemic ventricle
◦ Increase cardiac output
● Cyanosis may result from rt—>lt shunt
53
54. Fenestrated Fontan…
● Fenestrations decrease postop pleural effusions
● May be closed after patients adapt to new
hemodynamics
● Now, fenestrations are seldom created during
completion of Fontan
◦ Improved patient selection and preparation
◦ Improved staging
54
55. FONTAN PHYSIOLOGY
● Man-made neoportal system, where one
capillary bed pools blood into another
capillary bed without receiving energy
from a pump
● Critical bottleneck within this circulation
● Output through a bottleneck is
determined by
◦ Pressure just above (systemic veins)
and below (pulmonary veins) the
bottleneck
◦ Resistance within the bottleneck (PVR)
55
57. Role of ventricle in Fontan circuit
● No longer controls CO
● Nor decreases extent of congestion in systemic veins
● Deteriorates over time by increasing its EDP
57
58. Volume overloading of ventricles…
● Throughout Fontan strategy, ventricle exposed to different and
extreme loading conditions
● During fetal life+ initial palliation: SV is volume overloaded (250%–
350% for BSA)
● At time of Glenn shunt: Volume load on SV is reduced to about 90%
for BSA
● At completion of Fontan circuit: Reduces down to 50%–80% for
BSA
58
59. Use of ACE inhibition in Fontan patients
● Enalapril or placebo was given for 10 weeks in 18 pts.
approx. 14 yrs after Fontan operation
● Tendency to worsen exercise performance
● Reduced incremental cardiac index during exercise in
the patients receiving enalapril
59
60. ACE -S:
● Many patients continue to receive ACE
inhibition, in hope of a beneficial effect when
given chronically
● It is possible that there are subgroups that may
benefit e.g. severe systolic dysfunction
● Presently no evidence for this therapy being
beneficial
60
61. Exercise and Fontan circulation
● Normal individuals increase their PBF
significantly during peak exercise
● Reduction in PVR
◦ Vasodilation and
◦ Recruitment of segments
● Increased RV work
◦ Flow acceleration
◦ Increased systolic pressures up to 70 mm Hg
61
63. Exercise cont…
● PA flow in Fontan is relatively low velocity+ laminar
● Different to normal pulsatile flow of PV bed in normal
circulation
● Release of NO from endothelium is dependent on
pulsatile flow in the normal circulation
● Experimentally, reducing pulsatility leads to reduced NO
production and an increase in vascular resistance
63
64. Good Fontan…
● Growth and development of PAs during infancy
prior to Glenn shunt crucial
● Initial palliative procedure (Banding/ shunt): Most
important intervention and determinant of good
Fontan haemodynamics
64
66. ● Independent predictors of mortality:
1. Pre-op high PA pressure
2. Severe infection in early post-op period
3. Pleural and pericardial effusion
4. Low cardiac output
5. Sinus node injury
6. Pulmonary and systemic venous obstruction
66
67. Factors associated with long term morbidity
1. Progressive ventricular dysfunction
2. Systemic venous hypertension
3. RA distension (Classical)
4. Thromboembolic episodes
5. Worsening cyanosis
6. Heterotaxy syndromes
7. Significant AV valve regurgitation
8. NYHA class III / IV (pre-op)
9. Elevated PA pressure (pre-op)
67
68. TREATMENT OF CIRCULATORY
FAILURE :
● Treatment strategies:
◦ Open up bottleneck (decrease impedance of neoportal
system)
◦ Bypass bottleneck (fenestration)
◦ Increase pressure before bottleneck (systemic venous
pressure)
◦ Enhance run-off after bottleneck (ventricular suction)
68
69. Increase in Systemic Venous Pressures
● Temporarily increase cardiac output during
exercise (up to 30 mm Hg)
● Chronic high venous pressures in excess of 18–
20 mm Hg: Poorly tolerated
● Results in
◦ Congestion (Oedema, ascites, lymphatic failure)
◦ Progressive veno-venous collaterals with cyanosis
● Use of Diuretics: Worsens preload
69
70. Decrease in impedance in neoportal system
● Conversion to Cavo-pulmonary connection (in
older circuits)
● Early detection: Management of focal areas of
stenosis, hypoplasia, distortion or excessive
collateral flow
● Regular exercise and adapted breathing patterns
(lowers PVR by vessel recruitment+
vasodilation)
70
71. Breathing…
● Work of breathing is a significant additional
energy source to circulation in Fontan
● Normal negative pressure inspiration has been
shown to increase PBF after atrial pulmonary
connection and TCPC
71
72. Breathing…
● Philadelphia group using magnetic resonance
flow measurements: Estimated that
approximately 30% of CO can be directly
attributed to work of breathing in patients after
TCPC
72
73. PPV…
● Increasing levels of PEEP during positive pressure
ventilation is adverse to Fontan circulation
● Higher the MAP: Lower the CI
● Maintain with minimum mean airway pressure
compatible with normal oxygenation and ventilation
73
76. Ventricular suction
● No lusiotropic drugs available currently
● Agents which alter contractility, heart rate or
afterload: Negligible effects
76
77. Fenestration
● Proven to improve cardiac output and reduce
congestion
● Closing fenestration: Improved oxygen
saturations both at rest+ during exercise
● Secondary creation of a fenestration late in a
failing but ‘pink’ Fontan: Not well tolerated
● Percutaneous fenestration may have a role while
awaiting cardiac transplant
77
81. LV…
● Manifests as exercise intolerance
● Causes of LV dysfunction :
◦ Altered mechanics of ventricle in fontan circulation
◦ Congenital malformation: Predispose to ventricular dysfunction
◦ Previous surgical interventions
◦ Morphologic RV or an indeterminate primitive ventricle
◦ AVVR
81
82. Pulmonary Circulation
● PAs: Morphologically abnormal (small,
discontinuous or stenosed)
● Stenosis or leakage of surgical anastomoses:
Adverse effect on PBF
82
83. Collateral Vessels And Shunts
● Increased risk for formation of pulmonary arteriovenous
malformations
● Significant rt—> lt shunts+ cyanosis
● Causes of cyanosis:
1. Patent collateral vessels between systemic veins and pulmonary
veins
2. Patent systemic veins that extend directly into the left atrium
3. Incomplete closure of ASD
4. Redirection of coronary sinus to LA
5. Fenestration fontan
83
84. Shunts…
● Left-to-right shunts :
◦ Aortopulmonary collateral vessels
● Results in
◦ Volume overload of the SV
◦ Increased PBF and PA pressure
84
85. Coagulation abnormalities
● Reason :
◦ Dilated atrium
◦ Low cardiac output
◦ Coagulation abnormalities associated with hepatic
congestion
◦ Chronic cyanosis induced polycythemia
◦ Protein C+S and antithrombin III deficiency(liver
dysfunction)
◦ Increased platelet reactivity
◦ PLE
● Altered balance b/w pro and anti-coagulants
85
86. Coagulation abnormalities…
● Increased frequency of pulmonary
thromboembolic events
● Venous thromboembolism: 3%–16%
● Stroke: 3%–19%
● Massive pulmonary embolism: MCC of sudden
out-of-hospital death
86
87. Anticoagulation…
● No consensus on post op mode and duration of prophylactic
anticoagulation
● Aspirin: Uncomplicated pts
● Full anticoagulation
◦ Previous thrombi
◦ Spontaneous ECHO contrast
◦ Arrhythmias
◦ Dilatation of atrial / venous structures
◦ PLE
● Protocol in Star Hospital:
◦ Oral anti platelet lifelong: Aspirin 5mg/kg/day
87
88. Severe Hypoxemia (Post-fontan)
● Pts with fontan: Baseline SpO2 94+2%
● If severe desaturation: Rule out:
◦ Large fenestration
◦ Intrapulmonary AV fistulae
◦ Abnormal venovenous collaterals
● If present needs percutaneous occlusion
88
89. Functional Status+Exercise Tolerance
● Most patients lead a nearly normal life, including
mild to moderate sport activities
● >90% of all hospital survivors are in NYHA1/ 2
● Do well educationally+ pursue variety of
professions
● In time, progressive decline of functional status
in some subgroups
89
90. Arrhythmia
● Incidence:10-40% upto10 yrs after surgery
● Heterotaxy syndromes are prone for rhythm disorders
● Commonest arrhythmia :
◦ Sinus node dysfunction: 13-16%
◦ Intra-atrial re-entry or atrial flutter
●Refractory to anti arrhythmics
●Deteriorates quickly into cardiac failure
●Full Anticoagulation
●Conversion to Extracardiac fontan
◦ Ventricular arrhythmias: Rare
90
91. Lymphatic System
● Fontan circulation operates beyond the functional
limits of lymphatic system
● Affected by high venous pressure and impaired
thoracic duct drainage
● Leads to :
◦ Interstitial pulmonary edema or lymphedema
◦ Pericardial effusions
◦ Pleural effusions
◦ Chylothorax (peri-op)
91
92. Protein-Losing Enteropathy
● Relatively uncommon manifestation of failing Fontan
circulation
● Most frequent lymphatic problem in long term follow up
● Cause unclear
● Intestinal lymphangiectasia with leakage of lymphocytes,
chylomicrons and serum proteins
92
93. PLE…
● Loss of enteric protein may be due to elevated systemic
venous pressure that is transmitted to hepatic circulation
● Lead to hypoproteinemia, immunodeficiency, hypocalcemia,
and coagulopathy
● Clinical features :
◦ Edema
◦ Ascites
◦ Immunodeficiency
◦ Fatiguability
◦ Hypocalcemia
93
94. Treatment Options For PLE
● Diet :
◦ High in calories
◦ High protein content
◦ Medium chain triglyceride fat supplements
◦ Low salt
● Diuretics
● Protein infusions
94
95. PLE TREATMENT…
● Corticosteroids, heparin and octreotide have
been tried
● Resection of most affected part of gut
● Cardiac transplantation with immunosuppressive
therapy
95
96. PLE Cont...
● PLE: Relatively rare complication
● In an international multicentre study involving 35 centres
and 3029 patients with Fontan repair between 1975 and
1995, PLE occurred in 114 patients: 3.8%
● Very poor prognosis
● 5y survival:59%
96
97. Plastic Bronchitis
● Rare but serious complication
● 1%–2% of patients
● Noninflammatory mucinous casts form in
tracheobronchial tree and obstruct the airway
● Exact cause: Unknown
● May lead to development of lymphoalveolar fistula
and bronchial casts
97
98. Plastic bronchitis…
● Clinical features :
◦ Dyspnea
◦ Cough
◦ Wheezing
◦ Expectoration of casts
◦ Severe respiratory distress with asphyxia,
cardiac arrest, or death
98
99. Plastic bronchitis cont…
● Treatment :
◦ Medical management difficult
◦ Repeated bronchoscopy to remove thick casts
◦ Surgical ligation of thoracic duct
99
100. Reproduction/Pregnancy
● Most females have normal menstrual patterns
● Problems in pregnancy :
◦ Right heart failure
◦ Risk of Right-to-left shunt: Decrease in arterial saturation
◦ Increased risk for venous thrombosis and pulmonary embolus
● Successful pregnancy: Rare but possible
● SpO2 < 85%: Predictive of increased risk
● Risk of CHD in fetus: Unknown
100
101. POST-OP EVALUATION
● Clinical assessment
● Lab: CBC, LFTs, PT/INR
● ECG
● ECHO with color and tissue doppler
● Holter monitoring
● Cardiac MRI – best modality
● Cardiac catheterization
101
102. CARDIAC TRANSPLANTATION
● Failing Fontan circulation can benefit from
orthotopic cardiac transplantation
● Main indications for transplantation:
◦ Heart failure
◦ Intractable arrhythmias
◦ Protein-losing enteropathy
◦ Plastic bronchitis
102
103. Post transplant…
● Prognosis worse in Fontan failure than for other
CHDs
● PLE: Reported to resolve in patients who survive
for >1 mth after transplantation
● Predictors of morbidity and mortality :
◦ Age at operation
◦ Anatomic substrate for fontan operation
103
104.
105. The landmark paper…
● All patients having undergone Fontan surgery and
follow-up at Children’s Hospital Boston were included if
they were born before January 1, 1985, and lived
● Type of Fontan surgery was classified into the following
4 categories:
◦ RA–to–PA anastomosis
◦ RA–to–RV connection
◦ Intraatrial lateral tunnel (LT)
◦ Extracardiac conduit (ECC)
105
106. The landmark paper cont…
● A total of 261 patients, 121 female (46.4%)
● Had their first Fontan surgery at a median age of 7.9
years
● 33 (12.6%) of which were fenestrated
● Median follow-up of 12.2 years
● Type of first Fontan:
◦ RA-PA connection:135 (51.7%)
◦ RA-RV: 25 (9.6%)
◦ LT: 98 (37.5%)
◦ ECC: 3 (1.1%)
106
107.
108. Perioperative Mortality
● Of 52 perioperative deaths, 41 (78.9%) were early and
11 (21.1%) were late
● Importantly, perioperative mortality rates decreased
steadily over time
● First Fontan surgery:
◦ Before 1982: 36.7%
◦ 1982 to1989:15.7%
◦ 1990 or later: 1.9%
108
109. Long-Term Survival
● Actuarial event-free survival rates at 1, 10, 15, 20, and 25
years were 80.1%, 74.8%, 72.2%, 68.3%, and 53.6%
● Significant disparities b/w Fontan categories mainly due
to peri-op deaths in an earlier surgical era
● In peri-op survivors, freedom from death or cardiac
transplantation was comparable among all types
109
110. Death resulting from thromboembolism
● Occurred at a median age of 24.9 years
● 8.7 years after Fontan surgery
● Actuarial freedom from thromboembolic death was
98.7% at 10 years and 90.8% at 25 years
● All patients had RA-PA Fontan surgeries except for 1
patient with an LT
110
111. The landmark paper cont…
● Predictors of Thromboembolic Death in
Perioperative Survivors :
◦ Atrial fibrillation
◦ Lack of aspirin or warfarin therapy
◦ Thrombus within Fontan
111
112. Heart Failure
● Heart failure related deaths occurred at mean age of 22.9
● 4.3 years after Fontan surgery
● Actuarial freedom from death caused by heart failure was
99.5% at 10 yrs and 95.8% at 25 yrs
● Risk factors were single RV morphology, higher
postoperative RA pressure, and protein-losing
enteropathy
112
113. Conclusions of the paper
● Leading cause of death was perioperative, particularly in
an earlier era
● Gradual attrition was noted thereafter, predominantly
from thromboembolic, heart failure related, and sudden
deaths
● 70% actuarial freedom from all-cause death or cardiac
transplantation at 25 years
113
114. Take home message…
● Palliative and not curative
● Long term follow up necessary
114