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.
Incidental cor triatriatum sinister with congenital mr inAhmad Y. Alansi
油
- This case report describes a rare case of cor triatriatum sinister (extra membrane dividing the left atrium into two chambers) with congenital mitral regurgitation in a 25-year-old female patient.
- Echocardiography revealed an anomalous fibromuscular membrane dividing the left atrium into superior and inferior chambers, as well as a dilated annulus, hypoplastic leaflets, and chordal abnormalities leading to severe mitral regurgitation.
- At surgery, the membrane was excised and mitral valve replacement was performed with a mechanical prosthesis. The patient recovered well post-operatively.
- Cor triatriatum with congenital mitral
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
segment approach to congenital heart diseasesSumiya Arshad
油
The Van Praagh classification system uses a "S, D, S" notation to systematically analyze congenital heart defects based on 1) visceroatrial situs, 2) ventricular loop orientation, and 3) position of the great arteries. This facilitates communication between physicians by providing a standardized approach to describing abnormalities in cardiac chamber position, connections, and vessel arrangements.
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
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.
Congenitally corrected transposition of great arteriesDheeraj Sharma
油
This document provides an overview of congenitally corrected transposition of the great arteries (CCTGA). Key points include:
- CCTGA is a rare congenital heart defect where the ventricles are transposed but the atria are connected to the physically opposite ventricles, resulting in circulatory pathways in series.
- Patients may be asymptomatic for years but eventually develop right ventricular failure or left ventricular outflow tract obstruction. Diagnosis is made through physical exam, chest x-ray, and electrocardiogram showing right ventricular hypertrophy.
- Associated anomalies include ventricular septal defects, pulmonary stenosis, Ebstein's anomaly of the tricuspid valve, and heart block. Surgical
Congenital Heart Disease An Approach for Simple and Complex AnomaliesNizam Uddin
油
This document provides an overview of congenital heart disease for an exam, including:
- The most common defects like ventricular septal defects and tetralogy of Fallot.
- Case examples are presented and questions ask the reader to identify defects and complications.
- Surgical procedures for defects are discussed like arterial switch operation for transposition of the great arteries.
- Imaging views of common defects are shown and labeled.
The document serves as a study guide for the ASCeXAM by reviewing common congenital heart conditions and surgical treatments. Imaging examples help identify defects.
This document discusses atrioventricular canal defects (AVSDs), including their embryogenesis and pathophysiology. It describes the anatomy and classification of partial and complete AVSDs. Partial AVSDs involve a primum atrial septal defect with two distinct but contiguous AV valves, while complete AVSDs have a single common AV valve. The embryogenesis of AVSDs involves faulty development of the endocardial cushions. The document provides detailed descriptions and images of the anatomy and features of partial and complete AVSDs. It discusses the clinical aspects of AVSDs including prevalence, association with Down syndrome, surgical repair outcomes, and lifelong surveillance needs.
Echocardiographic Evaluation of LV Diastolic FunctionJunhao Koh
油
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.
- 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.
1) Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac anomaly where the connections of the great arteries and ventricles are discordant. The right ventricle functions as the systemic ventricle and the tricuspid valve is the systemic atrioventricular valve.
2) The right ventricle is not well-suited to function as the systemic ventricle long-term, often leading to ventricular dysfunction, tricuspid regurgitation, and heart failure. Management options include physiologic repair, anatomic repair such as the double switch operation, or a Fontan pathway.
3) The ideal surgical approach remains debated and depends on the individual
This document discusses and provides instructions for determining whether a heart has a D-loop or L-loop configuration based on the positioning of the right and left ventricles. It instructs the reader to use their right hand to model the right ventricle, with the thumb as the inlet and fingers as the outlet, to identify a D-loop if it fits correctly. For an L-loop, it instructs using the left hand instead to model the right ventricle, with the thumb as the inlet and fingers as the outlet, to identify an L-loop if it fits correctly. In both cases, it notes that looping refers to the relationship between the positions of the left and right ventricles.
This document discusses the history, diagnosis, and treatment of transposition of the great arteries (TGA). It notes that TGA is a congenital heart defect where the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. The document outlines the key developments in the surgical treatment of TGA, from early septostomies and shunts to the arterial switch procedure. It also describes the clinical presentation and management of different variations of TGA.
This document describes two cases of tetralogy of Fallot with absent pulmonary valve.
Case 1 is an 8 year old male with central cyanosis, recurrent respiratory infections and shortness of breath. Echocardiogram showed large subaortic VSD, absent pulmonary valve, and non-confluent pulmonary arteries.
Case 2 is a 15 year old male diagnosed with CHD at age 1. He has cyanosis, SOB, and clubbing. Echocardiogram showed large subaortic VSD, absent pulmonary valve, narrow pulmonary annulus, and confluent aneurysmal pulmonary arteries.
Both patients underwent catheterization which confirmed the echocardiogram findings and showed significant right ventricular
Development of inf venacava and pulmonary veinsanuppslides
油
1. The inferior vena cava forms from remnants of the right posterior cardinal vein, right supracardinal vein, and connections between the right subcardinal and hepatocardiac veins.
2. The suprarenal veins are derived from remnants of the subcardinal veins above the inter-subcardinal anastomosis, and the gonadal veins are derived from remnants below the inter-subcardinal anastomosis.
3. The termination of the right suprarenal vein in the inferior vena cava and the left suprarenal vein in the left renal vein results from their developmental origins in the subcardinal veins.
Initially, the pulmonary vein opens as a single vessel into
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
segment approach to congenital heart diseasesSumiya Arshad
油
The Van Praagh classification system uses a "S, D, S" notation to systematically analyze congenital heart defects based on 1) visceroatrial situs, 2) ventricular loop orientation, and 3) position of the great arteries. This facilitates communication between physicians by providing a standardized approach to describing abnormalities in cardiac chamber position, connections, and vessel arrangements.
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
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.
Congenitally corrected transposition of great arteriesDheeraj Sharma
油
This document provides an overview of congenitally corrected transposition of the great arteries (CCTGA). Key points include:
- CCTGA is a rare congenital heart defect where the ventricles are transposed but the atria are connected to the physically opposite ventricles, resulting in circulatory pathways in series.
- Patients may be asymptomatic for years but eventually develop right ventricular failure or left ventricular outflow tract obstruction. Diagnosis is made through physical exam, chest x-ray, and electrocardiogram showing right ventricular hypertrophy.
- Associated anomalies include ventricular septal defects, pulmonary stenosis, Ebstein's anomaly of the tricuspid valve, and heart block. Surgical
Congenital Heart Disease An Approach for Simple and Complex AnomaliesNizam Uddin
油
This document provides an overview of congenital heart disease for an exam, including:
- The most common defects like ventricular septal defects and tetralogy of Fallot.
- Case examples are presented and questions ask the reader to identify defects and complications.
- Surgical procedures for defects are discussed like arterial switch operation for transposition of the great arteries.
- Imaging views of common defects are shown and labeled.
The document serves as a study guide for the ASCeXAM by reviewing common congenital heart conditions and surgical treatments. Imaging examples help identify defects.
This document discusses atrioventricular canal defects (AVSDs), including their embryogenesis and pathophysiology. It describes the anatomy and classification of partial and complete AVSDs. Partial AVSDs involve a primum atrial septal defect with two distinct but contiguous AV valves, while complete AVSDs have a single common AV valve. The embryogenesis of AVSDs involves faulty development of the endocardial cushions. The document provides detailed descriptions and images of the anatomy and features of partial and complete AVSDs. It discusses the clinical aspects of AVSDs including prevalence, association with Down syndrome, surgical repair outcomes, and lifelong surveillance needs.
Echocardiographic Evaluation of LV Diastolic FunctionJunhao Koh
油
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.
- 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.
1) Congenitally corrected transposition of the great arteries (ccTGA) is a rare cardiac anomaly where the connections of the great arteries and ventricles are discordant. The right ventricle functions as the systemic ventricle and the tricuspid valve is the systemic atrioventricular valve.
2) The right ventricle is not well-suited to function as the systemic ventricle long-term, often leading to ventricular dysfunction, tricuspid regurgitation, and heart failure. Management options include physiologic repair, anatomic repair such as the double switch operation, or a Fontan pathway.
3) The ideal surgical approach remains debated and depends on the individual
This document discusses and provides instructions for determining whether a heart has a D-loop or L-loop configuration based on the positioning of the right and left ventricles. It instructs the reader to use their right hand to model the right ventricle, with the thumb as the inlet and fingers as the outlet, to identify a D-loop if it fits correctly. For an L-loop, it instructs using the left hand instead to model the right ventricle, with the thumb as the inlet and fingers as the outlet, to identify an L-loop if it fits correctly. In both cases, it notes that looping refers to the relationship between the positions of the left and right ventricles.
This document discusses the history, diagnosis, and treatment of transposition of the great arteries (TGA). It notes that TGA is a congenital heart defect where the aorta arises from the right ventricle and the pulmonary artery from the left ventricle. The document outlines the key developments in the surgical treatment of TGA, from early septostomies and shunts to the arterial switch procedure. It also describes the clinical presentation and management of different variations of TGA.
This document describes two cases of tetralogy of Fallot with absent pulmonary valve.
Case 1 is an 8 year old male with central cyanosis, recurrent respiratory infections and shortness of breath. Echocardiogram showed large subaortic VSD, absent pulmonary valve, and non-confluent pulmonary arteries.
Case 2 is a 15 year old male diagnosed with CHD at age 1. He has cyanosis, SOB, and clubbing. Echocardiogram showed large subaortic VSD, absent pulmonary valve, narrow pulmonary annulus, and confluent aneurysmal pulmonary arteries.
Both patients underwent catheterization which confirmed the echocardiogram findings and showed significant right ventricular
Development of inf venacava and pulmonary veinsanuppslides
油
1. The inferior vena cava forms from remnants of the right posterior cardinal vein, right supracardinal vein, and connections between the right subcardinal and hepatocardiac veins.
2. The suprarenal veins are derived from remnants of the subcardinal veins above the inter-subcardinal anastomosis, and the gonadal veins are derived from remnants below the inter-subcardinal anastomosis.
3. The termination of the right suprarenal vein in the inferior vena cava and the left suprarenal vein in the left renal vein results from their developmental origins in the subcardinal veins.
Initially, the pulmonary vein opens as a single vessel into
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 dellipertensione arteriosa polmonareguch-piemonte
油
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.
3. Classificazione
≒ RVPAT sopracardiaco, in vena anonima, VCSS o
VCSD (50%)
≒ RVPAT infra-cardiaco, in vena porta o VCI (20%)
≒ RVPAT intra-cardiaco, nel seno coronarico o atrio
destro (25%)
≒ RVPAT misto (5%)
4. Embriologia
≒ Stop embriologico. Mantenimento delle
connessioni tra vene polmonari e vene
sistemiche. Mancata connessione con latrio sx
5. ≒ Shunt dx-sx obbligato
≒ Cardiopatia DIA-dipendente
≒ Presenza o assenza di ostruzione al ritorno
venoso polmonare
6. Clinica
≒ Come un DIA ma cianosi +/≒ Soffio dolce da SVP relativa
≒ Polipnea etc
SE BLOCCATO
≒ Urgenza neonatale
≒ Bassa portata e edema polmonare
≒ Mortalit operatoria 25%
7. Clinica
≒ Dilatazione di AD, VD, polmonare e collettore
venoso
≒ Rx: cardiomegalia e iperafflusso
≒ ECG: sovraccarico dx (BBDi), e sovraccarico
atriale
8. Trattamento
≒ Forme extra-cardiache: anastomosi collettore
venoso e atrio sx, legatura della vena di
drenaggio, chiusura del DIA
≒ Forme intra-cardiache: deviazione del setto
interatriale
9. ECO
≒ Sovraccarico VD
≒ AS piccolo
≒ Dove sono le VP?? (nellintracardiaco
sembrano entrare in AS)
≒ Shunt obbligato dx-sx trans-DIA
≒ Velocit flusso trans-DIA
≒ Identificazione del collettore (se presente)
10. Anche nel feto
≒ Atrio sx piccolo,
sepimentazione
atriale, VSx piccolo
30. Classificazione
≒ In VCSD
≒ In AD
≒ In vena anonima
≒ In VCI
≒ Associato o meno a DIA
≒ Anomalia del ritorno di tutto il polmone dx in VCI
(sindrome della scimitarra)
≒..........
33. Clinica
≒ La clinica e i sintomi dipendono dal grado di shunt
sx-dx
≒ Nella sindrome della scimitarra i sintomi
dipendono:
≒ dal grado di ipoplasia polmonare destra
≒ dalla possibile ostruzione della vena scimitarra
≒ dalla presenza e dal grado di ipertensione
polmonare
≒ dalla frequente presenza di sequestro polmonare
34. Trattamento
≒ Se shunt piccolo astensione
≒ Se shunt importante: correzione
≒ RVPAP destro in VCS: deviazione delle VPdx in AS
≒ RVPAP destro in AD: deviazione del setto interatriale
≒ RVPAP in vena anonima: anastomosi del collettore allAS
≒ S. della scimitarra: se ben tollerata, astensione
37. Cor triatriatum
definizione
Latrio sinistro 竪 diviso da una membrana
in due parti, di cui una posteriore che
riceve lo sbocco delle vene polmonari,
laltra anteriore, che si apre verso il VS
attraverso la mitrale
La membrana ha un orifizio pi湛 o meno
grande, la cui dimensione determina la
gravit della patologia