This document provides information on Ebstein's anomaly, a rare congenital heart defect involving abnormal development of the tricuspid valve. It discusses the embryology, anatomy, physiology, clinical presentation and natural history. Key points include:
- Ebstein's anomaly results from a failure of the tricuspid valve leaflets to properly separate from the myocardium during development. This causes downward displacement of the valve and dilation of the right ventricle.
- Clinical presentations vary from fetal cyanosis to incidental murmurs later in life. Arrhythmias are common. Survival depends on severity but most children and adolescents have little disability.
- Long term outcomes are limited but available data shows around 15
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
This document discusses localization of accessory pathways using electrocardiography. It describes that accessory pathways can be located in eight anatomical positions along the tricuspid and mitral valve annuli. Several algorithms are proposed to determine the location based on delta wave polarity and amplitude in various leads. The most accurate is the Arruda approach, which uses step-wise analysis of delta wave characteristics in leads I, II, aVL, aVF and V1 to identify the specific accessory pathway location with 90% sensitivity and 99% specificity. Characteristic ECG patterns are presented that help localize right anteroseptal, right posteroseptal, left lateral and right free wall accessory pathways.
Low flow Low gradient severe aortic stenosisAnuj Mehta
1) Low flow, low gradient severe aortic stenosis can occur with both low and preserved ejection fraction. Dobutamine stress echocardiography is important to differentiate true from pseudo-severe stenosis.
2) For low ejection fraction, aortic valve replacement is recommended irrespective of symptoms or flow reserve.
3) For preserved ejection fraction, alternatives to ejection fraction like valvulo-arterial impedance and global longitudinal strain can help identify intrinsic myocardial dysfunction and predict outcomes. Aortic valve replacement may be better than medical management in these patients.
The document discusses implantable cardioverter defibrillators (ICDs), including:
- ICDs protect against dangerous ventricular arrhythmias by delivering shocks or antitachycardia pacing. They contain pacemakers for bradycardia pacing.
- ICD implantation indications include secondary prevention after cardiac arrest or unstable VT, and primary prevention for those with structural heart disease and reduced ejection fraction.
- Potential complications include bleeding, infection, and inappropriate shocks. Patients require regular device checks and lifestyle restrictions like avoiding magnetic fields.
Tachycardia discriminating algorithms and trouble shooting of ICDsRaghu Kishore Galla
- ICDs use algorithms to differentiate ventricular tachycardia (VT) from supraventricular tachycardia (SVT) in order to minimize inappropriate shocks.
- Key criteria include cycle length, stability, sudden onset, morphology matching, and AV association. Stability measures variability in cycle length while morphology compares shapes of sensed events to a template.
- Discrimination is challenging and no single criterion is perfect. Combining criteria improves accuracy, but incorrect diagnoses still occur. Programming must be tailored to individual patients and arrhythmia mechanisms.
Based on the size of the defect, perimembranous VSDs between 4-18 mm in diameter would be suitable for closure with the Amplatzer VSD occluder. The device size would need to be selected based on the actual defect size as assessed by echocardiography. Adequate rims around the defect are required but specifics on rim measurements are not provided in this document. Other factors such as indications for closure and no contraindications to the percutaneous approach would also need to be evaluated for a particular patient.
Ventricular septal defects (VSDs) are openings in the wall separating the ventricles of the heart. There are four main types classified by location: membranous, muscular, supracristal, and inlet VSDs. Echocardiography is useful for diagnosing VSDs and assessing their characteristics like location, size, and impact on cardiac function. VSDs range from small and asymptomatic to large defects causing heart failure or pulmonary hypertension. Surgical or catheter-based closure may be required for large VSDs.
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.
Major aortopulmonary collateral arteries (MAPCAs) are abnormal blood vessels that provide blood flow to the lungs in some patients with congenital heart defects. MAPCAs originate from the aorta or its branches and connect abnormally to the pulmonary arteries. They develop when normal pulmonary blood flow is obstructed in utero. MAPCAs vary in number, origin and path to the lungs. They may cause uneven blood flow within the lungs and become narrowed over time. The clinical presentation of patients with MAPCAs depends on the pattern of blood flow they provide.
This document discusses atrioventricular septal defects (AVSDs). It begins with epidemiology, noting a prevalence of 4-5% of congenital heart defects. It then covers embryology, anatomy, pathology, classification, clinical features, diagnosis and management. Key points include abnormal development of endocardial cushions leading to absence of AV septum and common atrioventricular valves. Clinical features include congestive heart failure in infancy. Diagnosis is made via echocardiogram showing absent AV septum. Surgical repair aims to close defects and preserve left AV valve competence.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly where the left coronary artery arises from the pulmonary artery instead of the aorta. 85% of cases occur during the first 2 months of life. Isolated anomalous origin of the left anterior descending or circumflex arteries is even rarer than ALCAPA.
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.
This document discusses the echocardiographic features used to evaluate Ebstein's anomaly of the tricuspid valve. It describes how to assess the displacement and morphology of the tricuspid valve leaflets, degree of tethering, and dilation of the cardiac chambers. Cut-off values are provided to define abnormalities. The document also reviews how to evaluate tricuspid regurgitation and the anatomy of the tricuspid valve annulus, chordae, and right ventricle outflow tract. Assessment of left ventricular function is also mentioned. Evaluation of Ebstein's anomaly by 2D, M-Mode, Doppler and 3D echocardiography is covered. Scoring systems for evaluating severity and prognosis are
- The SYNTAX trial compared outcomes of percutaneous coronary intervention (PCI) using drug-eluting stents versus coronary artery bypass grafting (CABG) for treating three-vessel or left main coronary artery disease.
- For the primary endpoint of major adverse cardiac and cerebrovascular events at 12 months, CABG was superior to PCI. However, outcomes were similar between treatments for patients with less complex disease as measured by low or intermediate SYNTAX scores.
- For patients with more complex disease and high SYNTAX scores, CABG had significantly fewer primary events than PCI at 12 months.
Echocardiographic Evaluation of LV Diastolic FunctionJunhao Koh
The document discusses methods for evaluating left ventricular diastolic function using echocardiography. It describes the four phases of diastole, parameters used to assess diastolic function including mitral inflow patterns, mitral annular tissue Doppler, pulmonary vein flow, left atrial size and the Tei index. Grades of diastolic dysfunction and approaches from ASE/EAE and Mayo Clinic are summarized. Continuous wave Doppler of aortic regurgitation is also presented as a noninvasive method to evaluate left ventricular relaxation.
This document provides information on Ebstein's anomaly, a rare congenital heart defect involving abnormal development of the tricuspid valve. It discusses the embryology, anatomy, physiology, clinical presentation and natural history. Key points include:
- Ebstein's anomaly results from a failure of the tricuspid valve leaflets to properly separate from the myocardium during development. This causes downward displacement of the valve and dilation of the right ventricle.
- Clinical presentations vary from fetal cyanosis to incidental murmurs later in life. Arrhythmias are common. Survival depends on severity but most children and adolescents have little disability.
- Long term outcomes are limited but available data shows around 15
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
This document discusses localization of accessory pathways using electrocardiography. It describes that accessory pathways can be located in eight anatomical positions along the tricuspid and mitral valve annuli. Several algorithms are proposed to determine the location based on delta wave polarity and amplitude in various leads. The most accurate is the Arruda approach, which uses step-wise analysis of delta wave characteristics in leads I, II, aVL, aVF and V1 to identify the specific accessory pathway location with 90% sensitivity and 99% specificity. Characteristic ECG patterns are presented that help localize right anteroseptal, right posteroseptal, left lateral and right free wall accessory pathways.
Low flow Low gradient severe aortic stenosisAnuj Mehta
1) Low flow, low gradient severe aortic stenosis can occur with both low and preserved ejection fraction. Dobutamine stress echocardiography is important to differentiate true from pseudo-severe stenosis.
2) For low ejection fraction, aortic valve replacement is recommended irrespective of symptoms or flow reserve.
3) For preserved ejection fraction, alternatives to ejection fraction like valvulo-arterial impedance and global longitudinal strain can help identify intrinsic myocardial dysfunction and predict outcomes. Aortic valve replacement may be better than medical management in these patients.
The document discusses implantable cardioverter defibrillators (ICDs), including:
- ICDs protect against dangerous ventricular arrhythmias by delivering shocks or antitachycardia pacing. They contain pacemakers for bradycardia pacing.
- ICD implantation indications include secondary prevention after cardiac arrest or unstable VT, and primary prevention for those with structural heart disease and reduced ejection fraction.
- Potential complications include bleeding, infection, and inappropriate shocks. Patients require regular device checks and lifestyle restrictions like avoiding magnetic fields.
Tachycardia discriminating algorithms and trouble shooting of ICDsRaghu Kishore Galla
- ICDs use algorithms to differentiate ventricular tachycardia (VT) from supraventricular tachycardia (SVT) in order to minimize inappropriate shocks.
- Key criteria include cycle length, stability, sudden onset, morphology matching, and AV association. Stability measures variability in cycle length while morphology compares shapes of sensed events to a template.
- Discrimination is challenging and no single criterion is perfect. Combining criteria improves accuracy, but incorrect diagnoses still occur. Programming must be tailored to individual patients and arrhythmia mechanisms.
Based on the size of the defect, perimembranous VSDs between 4-18 mm in diameter would be suitable for closure with the Amplatzer VSD occluder. The device size would need to be selected based on the actual defect size as assessed by echocardiography. Adequate rims around the defect are required but specifics on rim measurements are not provided in this document. Other factors such as indications for closure and no contraindications to the percutaneous approach would also need to be evaluated for a particular patient.
Ventricular septal defects (VSDs) are openings in the wall separating the ventricles of the heart. There are four main types classified by location: membranous, muscular, supracristal, and inlet VSDs. Echocardiography is useful for diagnosing VSDs and assessing their characteristics like location, size, and impact on cardiac function. VSDs range from small and asymptomatic to large defects causing heart failure or pulmonary hypertension. Surgical or catheter-based closure may be required for large VSDs.
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.
Major aortopulmonary collateral arteries (MAPCAs) are abnormal blood vessels that provide blood flow to the lungs in some patients with congenital heart defects. MAPCAs originate from the aorta or its branches and connect abnormally to the pulmonary arteries. They develop when normal pulmonary blood flow is obstructed in utero. MAPCAs vary in number, origin and path to the lungs. They may cause uneven blood flow within the lungs and become narrowed over time. The clinical presentation of patients with MAPCAs depends on the pattern of blood flow they provide.
This document discusses atrioventricular septal defects (AVSDs). It begins with epidemiology, noting a prevalence of 4-5% of congenital heart defects. It then covers embryology, anatomy, pathology, classification, clinical features, diagnosis and management. Key points include abnormal development of endocardial cushions leading to absence of AV septum and common atrioventricular valves. Clinical features include congestive heart failure in infancy. Diagnosis is made via echocardiogram showing absent AV septum. Surgical repair aims to close defects and preserve left AV valve competence.
Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly where the left coronary artery arises from the pulmonary artery instead of the aorta. 85% of cases occur during the first 2 months of life. Isolated anomalous origin of the left anterior descending or circumflex arteries is even rarer than ALCAPA.
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.
This document discusses the echocardiographic features used to evaluate Ebstein's anomaly of the tricuspid valve. It describes how to assess the displacement and morphology of the tricuspid valve leaflets, degree of tethering, and dilation of the cardiac chambers. Cut-off values are provided to define abnormalities. The document also reviews how to evaluate tricuspid regurgitation and the anatomy of the tricuspid valve annulus, chordae, and right ventricle outflow tract. Assessment of left ventricular function is also mentioned. Evaluation of Ebstein's anomaly by 2D, M-Mode, Doppler and 3D echocardiography is covered. Scoring systems for evaluating severity and prognosis are
- The SYNTAX trial compared outcomes of percutaneous coronary intervention (PCI) using drug-eluting stents versus coronary artery bypass grafting (CABG) for treating three-vessel or left main coronary artery disease.
- For the primary endpoint of major adverse cardiac and cerebrovascular events at 12 months, CABG was superior to PCI. However, outcomes were similar between treatments for patients with less complex disease as measured by low or intermediate SYNTAX scores.
- For patients with more complex disease and high SYNTAX scores, CABG had significantly fewer primary events than PCI at 12 months.
Echocardiographic Evaluation of LV Diastolic FunctionJunhao Koh
The document discusses methods for evaluating left ventricular diastolic function using echocardiography. It describes the four phases of diastole, parameters used to assess diastolic function including mitral inflow patterns, mitral annular tissue Doppler, pulmonary vein flow, left atrial size and the Tei index. Grades of diastolic dysfunction and approaches from ASE/EAE and Mayo Clinic are summarized. Continuous wave Doppler of aortic regurgitation is also presented as a noninvasive method to evaluate left ventricular relaxation.
This document discusses the treatment of pulmonary arterial hypertension (PAH), including:
- Approved PAH therapies such as endothelin receptor antagonists, phosphodiesterase-5 inhibitors, and prostanoids.
- The three main pathways involved in PAH pathogenesis.
- Treatment recommendations for PAH associated with congenital heart disease, including the use of PAH-specific therapies.
- Evidence that PAH-specific therapies can reduce mortality in patients with Eisenmenger syndrome.
- Lung transplantation is an option for patients with inadequate response to maximal PAH therapy.
H trattamento dell’ipertensione arteriosa polmonareguch-piemonte
This document discusses the treatment of pulmonary arterial hypertension. Pulmonary arterial hypertension has several underlying causes, with the most common being idiopathic pulmonary arterial hypertension at around 50% of cases. Medications are the primary treatment and work to dilate blood vessels and improve heart function. These include endothelin receptor antagonists, phosphodiesterase type-5 inhibitors, prostanoids, and soluble guanylate cyclase stimulators. In severe cases, lung transplantation may be considered.
1. Il Difetto Inter Atriale:
Indicazioni all’intervento, quando e come ?
Il parere del cardiologo pediatra interventista
G. Agnoletti
Citta’della Salute, Torino
gagnoletti@citta’dellasalute.to.it
Salo’, 5 Aprile 2013
3. •In genere sporadiche
•Talora forme familiari :
- BAV di 1° grado
- gene Nkx2.5
•Sindrome di Holt-Oram :
- rara (1/100 000 nati)
- trasmissione AD
- gene sul braccio lungo del cr 12
- anomalie degli arti superiori
Le comunicazioni interatriali
eziologia
10. Alla nascita:
•inversione dello shunt atriale
•abbassamento delle RVP
•aumento di QP
•aumento pressione AS
•shunt atriale sx-dx
Le comunicazioni interatriali
SEMPRE????
11. Si puo’ e deve sempre chiudere il DIA?
AT
Cardiopatia DIA - dipendente!
12. Si puo’ e deve sempre chiudere il DIA?
RVPAT
Cardiopatia DIA - dipendente!
13. Si puo’ e deve sempre chiudere il DIA?
Ebstein
14. Quali DIA si possono chiudere?
Quando la circolazione non e’ DIA - dipendente
Quando l’anatomia e’ favorevole
Quando il DIA si deve chiudere?
•Se sovraccario VD
•Se associato RVPAP
•Se associato CAV (cleft)
15. Quali DIA si possono chiudere?
•Quando la circolazione non e’ DIA - dipendente
•Quando l’anatomia e’ favorevole
16. Quali DIA si devono chiudere?
•se sovraccarico VD
•se associato RVAPP
•se associato CAV (cleft)
B32.tif
17. Fisiopatologia del DIA
Lo shunt sx-dx dipende
dalla compliance di VD
e VS
Sovraccarico volumetrico:
AD,VD,AP
Reattività arteriosa
polmonare :
Normale nel bambino
18. Evoluzione
• Per il DIA piccolo possibile chiusura
spontanea
• Aritmie atriali (FA, flutter) dopo i 30 anni
• Disfunzione TARDIVA del VD, favorita
dalle aritmie
• Ipertensione polmonare nel 10-30%
19. ECO
• La diagnosi di DIA è posta
all’ECO
• L’ECO deve rispondere alle
seguenti domande
• Tipo di DIA e lesioni
associate
• Sovraccarico VD
• Modalità di chiusura:
anatomia
20. DIA ostium secundum
• Bambino: ECO 2D
• Proiezione subxifoidea,
parasternale asse corto,
apicale 4 cavità
• Adulto: TEE
22. DIA ostium secundum
ECO
•Sovraccarico VD
•Setto paradosso
•M-Mode o 2D: VD/VS > 0,7
•Ipertensione polmonare (IT, IP, curva settale)
•Misura del QP/QS : inutile e poco riproducibile
Un DIA senza sovraccarico VD deve essere
rispettato (sempre?)
23. DIA cavale superiore
• Bambino: ECO 2D in
subxifoidea
• Adulto: TEE cava-cava, 30°, 4
camere etc
• Difetto del SIA postero-
superiore
• RVPAP (VPD in VCSD)
30. And to close…
• Self centering devices
• Non self centering devices
• Proliferation of devices…..
• Evolution of old devices
• Same principles (cover and stent)
37. Limiti?
percutanea
7 mm5
mm
• Ampia gamma di diametri
• Cavo rigido, non si adatta all’anatomia
• Device rigido, non si adatta all’anatomia
• Introduttore con curva talora inadeguata
• Tasso basso di erosione (<0.01%)
• Ingombrante
45. PVD sovrasistemica?
Assenza di shunt….disfunzione VD…
• Terapia percutanea:
creazione di DIA x
evitare la sincope
(P=QxR)
• Prezzo?
Desaturazione (shunt
dx-sx)….
