TAPVC defines the anomaly in which the pulmonary veins have no connection with the left atrium. Rather, the pulmonary veins connect directly to one of the systemic veins (TAPVC) or drain in to right atrium.
A PFO or ASD is present essentially in those who survive after birth
When pulmonary veins drain anomalously into the right atrium either because of complete absence of the interatrial septum or malattachment of the septum primum , then it is known as total anomalous pulmonary venous drainage.
When some or all of the pulmonary veins drain anomalously in to RA or its tributaries without being abnormally connected, the terms partially anomalous pulmonary venous drainage (PAPVD) or totally anomalous pulmonary venous drainage (TAPVD) with normal pulmonary venous connections are used.
An overview of the normal embryological process of development of the Aortic arch and the clinically relevant anomalies of the aortic arch development. Ideal for Cardiology Fellows.
TAPVC defines the anomaly in which the pulmonary veins have no connection with the left atrium. Rather, the pulmonary veins connect directly to one of the systemic veins (TAPVC) or drain in to right atrium.
A PFO or ASD is present essentially in those who survive after birth
When pulmonary veins drain anomalously into the right atrium either because of complete absence of the interatrial septum or malattachment of the septum primum , then it is known as total anomalous pulmonary venous drainage.
When some or all of the pulmonary veins drain anomalously in to RA or its tributaries without being abnormally connected, the terms partially anomalous pulmonary venous drainage (PAPVD) or totally anomalous pulmonary venous drainage (TAPVD) with normal pulmonary venous connections are used.
An overview of the normal embryological process of development of the Aortic arch and the clinically relevant anomalies of the aortic arch development. Ideal for Cardiology Fellows.
Definition:
Also known as Hypoplastic Right Heart Syndrome (HRHS)
It is a rare congenital cardiac lesion characterized by heterogeneous right ventricular development, an imperforate pulmonary valve, and possible extensive ventriculocoronary connections.
It is a type of congenital cyanotic heart disease, a severe form of Tetralogy of Fallot (TOF)
Newborn patients present cyanotic with high desaturation and pulmonary blood flow that depend on patent ductus arteriosus
Tetralogy of Fallot
Tetralogy of Fallot with Pulmonary
Stenosis
TETRALOGY OF FALLOT WITH CONGENITAL PULMONARY ATRESIA
Tetralogy of Fallot with Absent Pulmonary Valve
Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic (undersized) or absent right ventricle.
Similar to Classification and pathophysiology of tapvc (20)
Title: Sense of Taste
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the structure and function of taste buds.
Describe the relationship between the taste threshold and taste index of common substances.
Explain the chemical basis and signal transduction of taste perception for each type of primary taste sensation.
Recognize different abnormalities of taste perception and their causes.
Key Topics:
Significance of Taste Sensation:
Differentiation between pleasant and harmful food
Influence on behavior
Selection of food based on metabolic needs
Receptors of Taste:
Taste buds on the tongue
Influence of sense of smell, texture of food, and pain stimulation (e.g., by pepper)
Primary and Secondary Taste Sensations:
Primary taste sensations: Sweet, Sour, Salty, Bitter, Umami
Chemical basis and signal transduction mechanisms for each taste
Taste Threshold and Index:
Taste threshold values for Sweet (sucrose), Salty (NaCl), Sour (HCl), and Bitter (Quinine)
Taste index relationship: Inversely proportional to taste threshold
Taste Blindness:
Inability to taste certain substances, particularly thiourea compounds
Example: Phenylthiocarbamide
Structure and Function of Taste Buds:
Composition: Epithelial cells, Sustentacular/Supporting cells, Taste cells, Basal cells
Features: Taste pores, Taste hairs/microvilli, and Taste nerve fibers
Location of Taste Buds:
Found in papillae of the tongue (Fungiform, Circumvallate, Foliate)
Also present on the palate, tonsillar pillars, epiglottis, and proximal esophagus
Mechanism of Taste Stimulation:
Interaction of taste substances with receptors on microvilli
Signal transduction pathways for Umami, Sweet, Bitter, Sour, and Salty tastes
Taste Sensitivity and Adaptation:
Decrease in sensitivity with age
Rapid adaptation of taste sensation
Role of Saliva in Taste:
Dissolution of tastants to reach receptors
Washing away the stimulus
Taste Preferences and Aversions:
Mechanisms behind taste preference and aversion
Influence of receptors and neural pathways
Impact of Sensory Nerve Damage:
Degeneration of taste buds if the sensory nerve fiber is cut
Abnormalities of Taste Detection:
Conditions: Ageusia, Hypogeusia, Dysgeusia (parageusia)
Causes: Nerve damage, neurological disorders, infections, poor oral hygiene, adverse drug effects, deficiencies, aging, tobacco use, altered neurotransmitter levels
Neurotransmitters and Taste Threshold:
Effects of serotonin (5-HT) and norepinephrine (NE) on taste sensitivity
Supertasters:
25% of the population with heightened sensitivity to taste, especially bitterness
Increased number of fungiform papillae
Ozempic: Preoperative Management of Patients on GLP-1 Receptor Agonists Saeid Safari
Preoperative Management of Patients on GLP-1 Receptor Agonists like Ozempic and Semiglutide
ASA GUIDELINE
NYSORA Guideline
2 Case Reports of Gastric Ultrasound
ARTIFICIAL INTELLIGENCE IN HEALTHCARE.pdfAnujkumaranit
Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, especially computer systems. It encompasses tasks such as learning, reasoning, problem-solving, perception, and language understanding. AI technologies are revolutionizing various fields, from healthcare to finance, by enabling machines to perform tasks that typically require human intelligence.
Pulmonary Thromboembolism - etilogy, types, medical- Surgical and nursing man...VarunMahajani
Disruption of blood supply to lung alveoli due to blockage of one or more pulmonary blood vessels is called as Pulmonary thromboembolism. In this presentation we will discuss its causes, types and its management in depth.
New Directions in Targeted Therapeutic Approaches for Older Adults With Mantl...i3 Health
i3 Health is pleased to make the speaker slides from this activity available for use as a non-accredited self-study or teaching resource.
This slide deck presented by Dr. Kami Maddocks, Professor-Clinical in the Division of Hematology and
Associate Division Director for Ambulatory Operations
The Ohio State University Comprehensive Cancer Center, will provide insight into new directions in targeted therapeutic approaches for older adults with mantle cell lymphoma.
STATEMENT OF NEED
Mantle cell lymphoma (MCL) is a rare, aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for 5% to 7% of all lymphomas. Its prognosis ranges from indolent disease that does not require treatment for years to very aggressive disease, which is associated with poor survival (Silkenstedt et al, 2021). Typically, MCL is diagnosed at advanced stage and in older patients who cannot tolerate intensive therapy (NCCN, 2022). Although recent advances have slightly increased remission rates, recurrence and relapse remain very common, leading to a median overall survival between 3 and 6 years (LLS, 2021). Though there are several effective options, progress is still needed towards establishing an accepted frontline approach for MCL (Castellino et al, 2022). Treatment selection and management of MCL are complicated by the heterogeneity of prognosis, advanced age and comorbidities of patients, and lack of an established standard approach for treatment, making it vital that clinicians be familiar with the latest research and advances in this area. In this activity chaired by Michael Wang, MD, Professor in the Department of Lymphoma & Myeloma at MD Anderson Cancer Center, expert faculty will discuss prognostic factors informing treatment, the promising results of recent trials in new therapeutic approaches, and the implications of treatment resistance in therapeutic selection for MCL.
Target Audience
Hematology/oncology fellows, attending faculty, and other health care professionals involved in the treatment of patients with mantle cell lymphoma (MCL).
Learning Objectives
1.) Identify clinical and biological prognostic factors that can guide treatment decision making for older adults with MCL
2.) Evaluate emerging data on targeted therapeutic approaches for treatment-naive and relapsed/refractory MCL and their applicability to older adults
3.) Assess mechanisms of resistance to targeted therapies for MCL and their implications for treatment selection
Anti ulcer drugs and their Advance pharmacology ||
Anti-ulcer drugs are medications used to prevent and treat ulcers in the stomach and upper part of the small intestine (duodenal ulcers). These ulcers are often caused by an imbalance between stomach acid and the mucosal lining, which protects the stomach lining.
||Scope: Overview of various classes of anti-ulcer drugs, their mechanisms of action, indications, side effects, and clinical considerations.
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Ve...kevinkariuki227
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
Prix Galien International 2024 Forum ProgramLevi Shapiro
June 20, 2024, Prix Galien International and Jerusalem Ethics Forum in ROME. Detailed agenda including panels:
- ADVANCES IN CARDIOLOGY: A NEW PARADIGM IS COMING
- WOMEN’S HEALTH: FERTILITY PRESERVATION
- WHAT’S NEW IN THE TREATMENT OF INFECTIOUS,
ONCOLOGICAL AND INFLAMMATORY SKIN DISEASES?
- ARTIFICIAL INTELLIGENCE AND ETHICS
- GENE THERAPY
- BEYOND BORDERS: GLOBAL INITIATIVES FOR DEMOCRATIZING LIFE SCIENCE TECHNOLOGIES AND PROMOTING ACCESS TO HEALTHCARE
- ETHICAL CHALLENGES IN LIFE SCIENCES
- Prix Galien International Awards Ceremony
These lecture slides, by Dr Sidra Arshad, offer a quick overview of physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar leads (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
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Flu Vaccine Alert in Bangalore Karnatakaaddon Scans
As flu season approaches, health officials in Bangalore, Karnataka, are urging residents to get their flu vaccinations. The seasonal flu, while common, can lead to severe health complications, particularly for vulnerable populations such as young children, the elderly, and those with underlying health conditions.
Dr. Vidisha Kumari, a leading epidemiologist in Bangalore, emphasizes the importance of getting vaccinated. "The flu vaccine is our best defense against the influenza virus. It not only protects individuals but also helps prevent the spread of the virus in our communities," he says.
This year, the flu season is expected to coincide with a potential increase in other respiratory illnesses. The Karnataka Health Department has launched an awareness campaign highlighting the significance of flu vaccinations. They have set up multiple vaccination centers across Bangalore, making it convenient for residents to receive their shots.
To encourage widespread vaccination, the government is also collaborating with local schools, workplaces, and community centers to facilitate vaccination drives. Special attention is being given to ensuring that the vaccine is accessible to all, including marginalized communities who may have limited access to healthcare.
Residents are reminded that the flu vaccine is safe and effective. Common side effects are mild and may include soreness at the injection site, mild fever, or muscle aches. These side effects are generally short-lived and far less severe than the flu itself.
Healthcare providers are also stressing the importance of continuing COVID-19 precautions. Wearing masks, practicing good hand hygiene, and maintaining social distancing are still crucial, especially in crowded places.
Protect yourself and your loved ones by getting vaccinated. Together, we can help keep Bangalore healthy and safe this flu season. For more information on vaccination centers and schedules, residents can visit the Karnataka Health Department’s official website or follow their social media pages.
Stay informed, stay safe, and get your flu shot today!
4. • All the four pulmonary veins connect anomalously to a
systemic venous tributary of the right atrium or to the
right atrium proper but have no connection to the left
atrium.
• An inter atrial communication is essential for survival
• Very rarely, atrial septum may be intact – mixing via VSD/
PDA
TAPVR
7. • TAPVC is rare, representing 1.5–2% of all congenital
cardiovascular abnormalities .
• In a population-based registry, TAPVC represented
1.5% of all congenital cardiovascular malformations
and had a prevalence of 6.8/100 000 live births
• Sex ratio with supra-diaphragmatic TAPVC is almost
equal.
• Male predominance (3:1) is seen in infra-
diaphragmatic type of TAPVC
Bharati S, Lev M. Congenital anomalies of the pulmonary veins. Cardiovasc Clin 1973;5:23
8. Associated anomalies
• 2/3 are isolated
• PDA- 10-15%
• Common in heterotaxy syndromes
• Associated conditions :-
TGA
TOF
Single ventricle
Truncus arteriosus
Tricuspid atresia
HLHS
CoA
Asplenia or polysplenia
9. HISTORICAL LANDMARKS
1798 - 1st description by Wilson
1942 - autopsy series by Brody et al
1950 - Friedlich - 1st diagnosis by cardiac catheterisation
1951 - Muller- 1st surgical correction ( closed technique)
1956 - Lewis & Varco – surgery by hypothermia & inflow occlusion(open heart)
1956 - Kirklin - surgery on CPB
1957 – Darling, Rothney & Craig- 1st classification
1961 - Sloan – 1st surgery for infra cardiac variety by hypothermia & a period of
circulatory arrest
1967 - Dillard- surgery with DHCA without CPB.
10. Embryology
• The respiratory system develops as an
evagination from the foregut at 26 days.
• The venous plexus surrounding the early lung
buds drains into the cardinal and umbilico-
vitelline veins - part of the splanchnic
(systemic) venous system.
11. (A) Lateral view of the embryo. The foregut (green) gives rise to two lung buds (LB, putative
lungs). A splanchnic vascular network (pale blue) can be observed surrounding the LBs. The
venous tributaries drain into the sinus venosus (SV) to which is connected the non-
lumenized pulmonary vein and the midpharyngeal endothelial strand (MPES) that runs in
front of the gut from the venous to the arterial pole.
12.
13.
14.
15. • A:At 27 to 29 days of gestation, the primordial lung buds are enmeshed by the vascular
plexus of the foregut (the splanchnic plexus). At this stage, there is no direct connection to
the heart. Instead, there are multiple connections to the umbilicovitelline and cardinal
venous systems. A small evagination can be seen in the posterior wall of the left atrium to
the left of the developing septum secundum
16.
17.
18. (B) The splanchnic plexus (indicated as the pale blue network covering the foregut
including the LB) is already connected to the endocardium of the primitive heart tube, by
means of the MPES.
In early developmental stages the route of drainage is from the pulmonary venous
plexus to the systemic veins via pulmonary-to-systemic venous connections.
(C) The endothelial anlage of the pulmonary veins has lumenized to become the
common pulmonary vein (CPV), allowing drainage of the splanchnic plexus not only
to the systemic veins but also into heart.
(D) The CPV grows and dilates, and becomes the main route of drainage of the
pulmonary venous blood.
19.
20. (E) Central drainage period. At this stage, the primitive pulmonary-to-venous
connections have regressed entirely. The route of drainage of the pulmonary venous
blood is now directly into the heart. (F, G) Bifurcations of the CPV will be incorporated
into the left atrium (LA), contributing to the body of the LA. Usually four separate
pulmonary venous ostia can be recognized, although variations occur.
21.
22.
23. TAPVR occurs as a result of developmental failure or early atresia of
common pulmonary vein when collateral channels for pulmonary venous
return are available in the form of primitive connections between the
pulmonary venous plexus and the systemic veins.
The type of TAPVR is classified according to the persistent collateral
channels from pulmonary to systemic veins as follows:
• Supracardiac type includes connections to the left innominate vein, the
superior vena cava, or the azygos vein;
• Cardiac type includes connections to the coronary sinus or directly to
the right atrium; and
• Infracardiac type includes connections below the diaphragm to the
inferior vena cava, the portal vein, the hepatic veins, or the ductus
venosus
24. Embryologic basis of totally anomalous pulmonary venous connections.
B: Totally anomalous pulmonary venous connection results from failure to establish a normal
connection between the pulmonary venous plexus and the common pulmonary vein before the
connections with splanchnic venous system have regressed
25.
26. Classification
• Based on
* Pathway of pulmonary veins to the right atrium
* Presence or absence of obstruction
* Nature of inter atrial communication.
27. CLASSIFICATIONS
• DARLINGS CLASSIFICATION
• NEILLS CLASSIFICATION
• SMITHS CLASSIFICATION
• BURROUGHS AND EDWARD CLASSIFICATION
• UK CHOWDHARY ET AL MIXED TAPVC
CLASSIFICATION
• HERLONGS CLASSIFICATION
28. • Type- I( a& b) Supracardiac : 50%
• Type II (a & b) Cardiac : 25%
• Type III Infra cardiac : 20%
• Type IV Mixed : 5%
Lab invest 1957;6:44
1
29. Supra cardiac-Type 1 a
• Most common(40%)
• 4 pv cc vertical
vein innominate
vein SVC
• Vertical vein pass
anterior to LPA & Left
bronchus
• When vv pass in b/w
obstruction ( vascular
vice)
30.
31.
32. Supracardiac- Type 1 b
• 4 pv cpv vertical
vein SVC- RA jn/
azygos
• More common in right
atrial isomerism, 90% of
whom have TAPVR
33. Cardiac- Type II a
• Coronary sinus type-
More common variant
• 4pv Cpv coronary
sinus RA
34. Type II b
• Right atrial type
• All 4 pulmonary veins
drain directly to the RA
35.
36. Infra cardiac- type III
• 4 pv cpv vertical
vein pass thro
esophageal hiatus(t 10)
antr to esophagus
portal vein (65%)/
ductus venosus/
hepatic vein / IVC
37.
38. Type IV - mixed
• A combination of any pathways
• Most common- supracardiac + cardiac
39.
40. 248 patients over 15 yrs
168 boys; 80 girls
1 day to 24 yrs
Supra cardiac- 54%
Cardiac - 32.2%
Infra cardiac—3.6%
Mixed - 10.1%
Obstructed- 20%
Mortality- 19%
(Indian Heart J 2001; 53:
754–760)
41. • Right common cardinal vein
– SVC
– Azygos vein
• Left common cardinal vein
– Left innominate vein
– Coronary sinus
• Umbilico-vitelline system
– Hepatic vein
– Ductus venosus
– Portal vein
MIXED TYPES
Connection to:
2
43. Total anomalous pulmonary venous drainage :diagnostic criteria and a new classification. Am J Dis Child 101:41,1961
4
44. Mixed TAPVR
• Category I: bilateral and symmetrical connections
(2+2)
• Category II : bilateral and asymmetrical
connections (3+1)
• Category III : bizarre anatomic variants
A suggested new surgical classification for mixed totally anomalous pulmonary venous connection. Cardiol Young.2007;17:342-53.
5
45. Category 1: 2+ 2
A to D, Group of patients with bilateral and symmetrical connections
(“22” pattern of drainage).
46. Category II :3+1
A to F, Group of patients with bilateral and asymmetrical connections (“31” pattern of drainage)
47. Category III-Bizarre anatomic variants
A to E, Group of patients of bizarre anatomic variants of mixed total anomalous pulmonary
venous connection.
48. Herlong and colleagues suggested complete description
of the TAPVC including-
• Level of connections-
supracardiac,cardiac,infracardiac or mixed.
• Presence or absence of obstruction
• Cause of obstruction-extrinsic,intrinsic or obstructive
atrial septal communication.
Herlong et.al.Ann Thorac Surg 69(suppl):S56,2000
6
50. Pathophysiology
• Fetal life: Negligible
consequence less
blood through lungs
and pulmonary vein
beginning of
pathological changes
• After birth : Obligatory
R L shunt
51. • None of the pulmonary veins connect normally to the left atrium, the
only source of blood to the left atrium is blood that is shunted from the
right atrium across the defect to the left side of the heart.
• The highly oxygenated blood from the lungs completely mixes with the
poorly oxygenated blood returning from the systemic circulation. This
causes an overload of the right atrium and right ventricle leading to
enlargement of chambers.
• The increased blood volume going into the lungs can lead to pulmonary
hypertension and pulmonary edema.
52. • TAPVC is incompatible with life unless there is
an associated defect present that allows for
shunting of blood from the highly pressured
right side of the heart.
• A patent foramen ovale or an ASD is usually
present.
53. LUNGS
Qp
LA
LVRV
RA
Qs
• Early onset & rapid progression of pulmonary vascular
changes
↑ muscularity & abnormal distal extension.
• Arterialisation of pulmonary veins
• Small thick walled PV.
• 20% show stenosis of individual PV or Confluence –
intimal fibrous hyperplasia
RA -enlarged & thick walled
RV- enlarged due to volume overload
LA- small due to absence of PV component
LV- Nl size & thickness; ↓ EDV due to IVS shift
PA- Enlarged
IAS- ASD/ PFO universal SIZE OF ASD
• Determines the
systemic
circulation.
• Usually large
adequate ASD/
PFO is present.
• A gradient of ≥
3 mmHg b/w
RA & LA
indicate that
the
communication
is restrictive.
54. Cardiac chambers
• RA -enlarged & thick walled
• RV- enlarged due to volume
overload
• LA- small due to absence of
PV component
• LV- Normal size & thickness;
↓ EDV due to IVS shift
• PA- enlarged
• IAS- ASD/ PFO universal
55.
56. Pathophysiological Changes
• Obligatory mixing
• Obligatory Right to left shunt at ASD level
• Shunt at PDA level if Present
• Increased Pulmonary Flow
• Decreased or Normal Systemic flow
• Increased PVR
• PVH
57. Microscopic anatomy
• Early onset & rapid progression of pulmonary
vascular changes
• ↑ Muscularity & abnormal distal extension.
• Arterialisation of pulmonary veins
• Small thick walled PV.
• 20% show stenosis of individual pv or confluance –
intimal fibrous hyperplasia
58. The factors that influence the pathophysiology
of TAPVC include
1. Obligatory mixing of pulmonary and
systemic blood
2. Size of the atrial septal defect
3. Obstruction of the anomalous connection
4. Associated anomalies
59.
60. 1. Obligatory mixing :
• The systemic blood flow must be provided from
right side of the heart
Inter atrial communication
Distensibility & compliance of both ventricles.
61.
62. Determinant of Systemic Oxygen
saturation
• Degree of mixing
– if unrestricted mixing of blood returning to RAEqualization
of O2 saturation in all 4 chambers
– If restricted mixing-fetal pattern of preferential streaming of
SVCTVRVPA(PDADTA if PDA patent) vs
IVCPFOLALVAO
– Hence if
• Supracardiac TAPVC (SPaO2>SaO2)
• Infracardiac TAPVC (SaO2>SPaO2)
• Qp:Qs- directly determines SaO2
– Higher Qp:Qs- higher the SaO2
• Oxygenation- hampered by developing pulmonary edema
63. 2. SIZE OF ASD
• Determines the systemic circulation.
• Usually large adequate ASD/ PFO is present.
• A gradient of ≥ 3 mmHg b/w RA & LA indicate
that the communication is restrictive.
64. 3. Obstruction of the connection
• The presence of obstruction along the
anomalous channel is the most significant
factor in the hemodynamics
• Can be anatomic or functional
65.
66.
67. Causes of Obstruction
• Supracardiac- Ia- 40% ; Ib- 65%
- Extrinsic obstruction
- Intrinsic vertical vein obstruction
• Cardiac- least common- 20%
- At the coronary sinus opening
• Infracardiac- almost always(95-100%)
- Narrow & long vertical vein at the esophageal hiatus
- Hepatic capillary resistance
• Restrictive ASD/PFO
68.
69. Pathophysiology of obstruction-Role of PFO/ ASD
Non restrictive
Low gradients across PFO/ASD
Low RAP
Low RV preloading
RV failure delayed
Restrictive
↑RAP
Gradient is more across PFO
Pulmonary edema
↑PAP& PVRI
RVF↓Qp & Cyanosis
71. Determinant of Systemic flow
• If shunting through PFO/ASD (LV maintains the
Systemic perfusion)
– Preload
• Obstructed TAPVC- reduced filling
• Restriction at PFO
• Small poorly compliant LA
• Impaired LV filling- compliance,bulge of IVS towards LV
– Pump-Impaired LV function
– Afterload-eg. Hypoxemia↓/↑SVRsystemic blood
flow↑/↓
• If PDA dependent systemic perfusion
– RV performs the work of systemic perfusion
– Depends on SVR vs PVR
– RV function
72. Determinant of PVR
• Normal physiological drop in PVR increase in PBF
• Obstructed Return↑pulmonary venous
pressure↑transudatePulmonary
EdemaHypoxemiaArteriolar constriction↑PVR
• Mild or no obstruction- increased blood flowshear
stressendothelial proliferation to encroach
lumenpulmonary vascular obstructioncyanosis
• Pulmonary blood flow (balance b/w PA pressure and PVR)
73. Effect of feeding
• Hemodynamic compromise in infra cardiac
type.
• Compression at esophageal hiatus
• Increase in GI and portal blood flow ↑
portal & pulmonary venous pressure
pulmonary oedema.
74. Pathophysiology of Non-obstructed
TAPVC without PDA
• Obligatory RL shunting at PFO/ASD
• RA pressure >LA pressure
• Hemodynamics mainly determined by
• PVR
• SVR
• Restriction at PFO/ASD
• Effect of changes in PVR
– Physiological fall in PVR post
natally↑Qp:Qsimproves SaO2
– Mechanism- ↓pvr↓RV afterload↓RV esv & ↑RV
ef↑RA pressure↑RV filling↑RV output(Frank
Starling Law)
75. Pathophysiology of non-obstructed
TAPVC without PDA cntd.
• Effect of changes in SVR
– Decreased SVR↓LV esv & ↑ LV EF↓LA
pressure↑rl Shunt↑SBF and ↓
PBFdecreased SaO2
– Increased SVR- opposite occurs
• Effect of size of PFO/ASD
– Non restrictive- little rise in RAP, adequate LV filling
achieved, systemic perfusion adequately maintained
– Restrictive -↑RAP to maintain adequate LV
filling↑RVVO↑Qp PVHblood returning to
↑RAPvicious cyclepulmonary edema and RV
failure
76. Pathophysiology of Non-obstructed
TAPVC with PDA
• PDA may affect the oxygen saturation in the
great arteries
• Depending on degree of mixing and
preferential streaming of blood & Variety of
TAPVR
77. Pathophysiology of obstructed TAPVC
with PDA
• Obstructed return Severe PVHPAHPA to
Ao Shunting via PDA
• Effects
– Prevents PAP from becoming supra systemic
– ↓PBF
– ↓pulmonary edema, ↓Qp:Qs, ↓Sao2
• Effects of hypoxemia- ↓SVR ↑RL shunting
via PDAfurther hypoxemiasympatho-
adrenal stimulation ↑SVR ↓RL shunt via
PFO/PDA ↓tissue perfusioncyanosis
78. Pathophysiology of obstructed TAPVC
without PDA
• Closure of PDA
– ↑PAP
– ↑PBF
– ↑Qp:Qs
– ↑Oxygenation
– Higher risk of pulmonary edema
79. Pathophysiology of Infracardiac TAPVC:
Role of Ductus venosus
• Blood bypasses the hepatic
sinusoids
• If ductus venosus widely
patent- no PVH/Pulmonary
Edema
• If Ductus venosus
closesincreased portal and
pulmonary venous
pressurePAH can develop
80. Inhaled O2
• If administered for cyanosis
without identifying TAPVC
– Risk of increased
pulmonary edema
• Increased PBF due to
pulmonary
vasodilation
• PDA
constrictionincrease
d PA pressurePE
Effect of PGE1
• 2 opposing effects
– ↑Qp-by pulmonary vasodilation
– ↓Qp- by keeping PDA patent
and RL shunting
• Final outcome depends which
effect predominated
• PGE1 especially helpful in
infradiaphragmatic TAPVC by
keeping the ductus venosus
patent
• Systemic vasodilation may
require inotropic support during
PGE1 administration
81. Natural history
• With obstruction, the lifespan is brief. Pulmonary edema and
right ventricular failure ensue within days to weeks of birth.
• Most infants die in the first few days or weeks of life and survival
for up to 3–4 months is exceptional .
• The natural history is unfavorable even in patients with
unobstructed.
• TAPVC, most of whom die within 3–6 months of birth; 75–90% of
symptomatic infants do not reach 1 year of life.
• Heart failure and infections are the major causes of mortality.
• The minority who present after the first year of life invariably
have a low pulmonary vascular resistance and a nonrestrictive
atrial septal defect.
82. Natural history
Long survivors :
• Large interatrial communication
• Non-obstructive pulmonary veins
• Low pulmonary vascular resistance
• Usually supradiaphragmatic TAPVC
83. Clinical features
TAPVC without Pulmonary Venous Obstruction
• Patients are usually asymptomatic at birth.
• Tachypnea and feeding difficulties are the initial
symptoms usually within first few weeks of life.
• Then infants have recurrent resp.tract infections and
failure to
thrive.
• Mild cyanosis is present because of adequate mixing of
blood.
• Gradually they develop right heart failure and
84. Clinical features cont.
• TAPVC with Pulmonary Venous Obstruction
• Tachypnea,tachycardia and cyanosis within few hours of
birth (usually did not appear in the first 12 hours of life).
• D/D Respiratory distress syndrome-symptoms within 12
hours of life.
• Dyspnea is severe because of marked pulmonary
venous congestion and cyanosis is marked because
of reduced pulmonary flow.
• If left untreated death may occur from pulmonary edema
and RV failure within few days or weeks of life.
85. Clinical features cont.
• Those who survive their first year almost always
have supradiaphragmatic connections,low
pulmonary vascular resistance and a nonrestrictive
atrial septal defect.
• When an infradiaphragmatic venous channel
traverses the esophageal hiatus feeding,crying and
straining cause additional compression that
aggravates the dyspnea and cyanosis
• (Lucas et.al. Am J
Roentgenol.86;561,1961)
• Newborns with infradiaphragmatic connections and
asplenia
may have major esophageal varices.
86. On examination
TAPVC without Pulmonary Venous Obstruction
• Mild cyanosis with features of CHF.
• Prominant precordium with left lower parasternal heave.
• S1 loud,S2 wide split and fixed with loud P2.RVS3 present.
• ESM 3-4/6 at upper sternal border ( ↑ pulmonary flow).
• PSM due to TR and MDM due to increased flow across TV may
be there.
87. On examination cont.
TAPVC with Pulmonary Venous Obstruction
• Clinical condition is grave with minimal cardiac
findings.
• Signs of PAH present.
• Apex impulse is of RV type.
• S1 normal,S2 closely split,P2 loud.
• A short systolic murmur due to pulmonary artery
dilatation.
• Liver is enlarged and tender.
Editor's Notes
The respiratory system develops as an evagination from the foregut at 26 days.
The venous plexus surrounding the early lung buds drains into the anterior cardinal and umbilico-vitelline veins, both of which are part of the splanchnic (systemic) venous system.
The anterior cardinal veins give rise to the superior vena cava, the coronary sinus, and the azygos vein, and the umbilico-vitelline veins later form the portal venous system. In normal circumstances, the common pulmonary vein develops as an outpouching from the dorsal left atrial wall, eventually fusing with the pulmonary venous plexus at 27 days' gestation. Shortly thereafter, the anterior cardinal and umbilico-vitelline venous channels normally undergo involution (Fig. 93-1).
The respiratory system develops as an evagination from the foregut at 26 days.
The venous plexus surrounding the early lung buds drains into the anterior cardinal and umbilico-vitelline veins, both of which are part of the splanchnic (systemic) venous system.
The anterior cardinal veins give rise to the superior vena cava, the coronary sinus, and the azygos vein, and the umbilico-vitelline veins later form the portal venous system. In normal circumstances, the common pulmonary vein develops as an outpouching from the dorsal left atrial wall, eventually fusing with the pulmonary venous plexus at 27 days' gestation. Shortly thereafter, the anterior cardinal and umbilico-vitelline venous channels normally undergo involution (Fig. 93-1).
classification with prognostic implication based on length of anomalous channel
EARLY DIASTOLE-SIPHONING R->L left coronary compressed by large PA SHAPE-LOW LV FUNCTIONAL RESERVE-EXERCISE PR,ENDOCARDITIS