A RARE CASE OF PULMONARY ATRESIA WITH INTACT VENTRICULAR SEPTUM, CONGENITALLY UNGUARDED TRICUSPID VALVE ORIFICE AND MASSIVE RIGHT-SIDED DILATION

Some patients with pulmonary atresia with an intact ventricular septum, mild to moderate right ventricular hypoplasia, and a patent infundibulum remain duct dependent on the flow of blood through the arterial duct despite adequate relief of the obstruction within the right ventricular outflow tract.The objective of our study was to review the risk factors for stenting of the patent arterial duct, or construction of a Blalock-Taussig shunt, in the patients with pulmonary atresia and an intact ventricular septum who remain duct-dependent following radiofrequency valvotomy and dilation of the imperforate pulmonary valve.We reviewed the data from 53 patients seen between November 1995 and December 2001. Of the 47 patients who survived, 6 required stenting of the patent arterial duct, while 4 needed construction of a modified Blalock-Taussig shunt to augment the flow of blood to the lungs at a mean of 7 plus or minus 5.7 days following the initial intervention. The remaining 37 patients required no additional procedures. We compared the findings in these two groups.The mean diameter of the tricuspid valve in the patients requiring early reintervention was 8.5 plus or minus 3.7 millimetres, giving a Z-score of −1.1 plus or minus 1.47, whilst those in the group without early reintervention had values of 10.7 plus or minus 2.2 millimetres, giving a Z-score of −0.58 plus or minus 1.18 (p equal to 0.003). No statistically significant differences were found in right ventricular morphology, McGoon ratio, or residual obstruction across the right ventricular outflow tract after decompression of the right ventricle.The diameter of the tricuspid valve, therefore, appears to be the only factor predicting the need for augmentation of flow of blood to the lungs. As just over one-fifth of our survivors required such augmentation, we hypothesize that stenting of the patent arterial duct may be performed as an integral part of primary transcatheter therapy in patients with pulmonary atresia and intact ventricular septum who have moderate right ventricular hypoplasia and a small tricuspid valve.

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Surgical closure of the tricuspid valve for pulmonary atresia, intact ventricular septum, and right ventricle to coronary artery communications

Pediatric Cardiology ◽

10.1007/bf02427050 ◽

1984 ◽

Vol 5 (3) ◽

pp. 221-223 ◽

Cited By ~ 30

Author(s):

J. Deane Waldman ◽

John J. Lamberti ◽

James W. Mathewson ◽

Lily George

Keyword(s):

Coronary Artery ◽

Right Ventricle ◽

Tricuspid Valve ◽

Pulmonary Atresia ◽

Ventricular Septum ◽

Intact Ventricular Septum ◽

Surgical Closure

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Clinicopathological correlation—pulmonary atresia with imperforate tricuspid valve and aneurysmal dilatation of ascending aorta

Cardiology in the Young ◽

10.1017/s1047951100001591 ◽

1993 ◽

Vol 3 (3) ◽

pp. 220-224

Author(s):

Rafael Hirsch ◽

Leon Gerlis ◽

Jane Somerville

Keyword(s):

Heart Failure ◽

Atrial Fibrillation ◽

Tricuspid Valve ◽

Pulmonary Atresia ◽

Ascending Aorta ◽

Ventricular Septum ◽

Clinicopathological Correlation ◽

Intact Ventricular Septum ◽

Aneurysmal Dilatation ◽

Unusual Form

AbstractA patient with the rare combination of an unusual form of pulmonary atresia with intact ventricular septum and a Marfan-like disease is presented. She was extremely cyanosed in childhood but improved after construction of a left peripheral shunt at the age of nine years. She deteriorated gradually after the onset of atrial fibrillation at the age of 33, the subsequent course being complicated by a stroke and, finally, by intractable heart failure and death at the age of 39.

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Successful multiple Percutaneous Interventions and Biventricular Surgical Repair in a Child with Pulmonary Atresia with Intact Ventricular Septum— A Case Report

Cardiovascular Journal ◽

10.3329/cardio.v13i1.50569 ◽

2020 ◽

Vol 13 (1) ◽

pp. 81-85

Author(s):

Nurun Nahar Fatema Begum ◽

Md Ferdousur Rahman Sarkar ◽

Md Ashfaque Ahemmed Khan ◽

Mohammad Nazmul Islam Bhuiyan

Keyword(s):

Right Ventricular ◽

Tricuspid Valve ◽

Ductus Arteriosus ◽

Pulmonary Atresia ◽

Ventricular Septum ◽

Military Hospital ◽

Base Line ◽

Intact Ventricular Septum ◽

Cardiac Catheterization Laboratory

Pulmonary atresia (PA) with intact ventricular septum is a rare, heterogeneous congenital heart defect with varying degrees of right ventricular and tricuspid valve hypoplasia and wide spectrum clinical features. Initial treatment at presentation is to establish systemic to pulmonary shunt or ductus stenting. The treatment options i.e. biventricular, one and half ventricular or one ventricular repair are often dictated by the degree of development of the tricuspid valve and right ventricle. X, 11 years old teenage girl got admitted to Combined Military Hospital (CMH) Dhaka at 5 months of age with severe cyanosis, respiratory distress, acidosis and shock. After immediate stabilization and work up, she was diagnosed as pulmonary atresia with intact ventricular septum, flapped atrial septal defect (ASD) and a small patent ductus arteriosus (PDA). On urgent basis, stenting of PDA done with 3.5 X 11 mm coronary stent at cardiac catheterization laboratory. Her SPO2 stepped up to 90 % from base line SPO2 from 50% following procedure. At 15 months of age she underwent intracardiac repair with short trans annular patch and ASD left open as a vent for RV. On follow up her ASD was getting larger and became desaturated and symptomatic. She was followed up for the right ventricular (RV) and tricuspid valve growth and ASD shunt. As her RV was developing nicely during follow up and achieved TV z score –1, ASD device closure was done with 18 mm Amplatzer ASD device at 5 years of age. She is now asymptomatic and thriving well. Cardiovasc. j. 2020; 13(1): 81-85

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Pulmonary atresia with intact ventricular septum: a multicenter analysis of 21 prenatal diagnosis cases

10.21516//2413-1458-2017-16-1-15-21 ◽

2017 ◽

Author(s):

M.V. Medvedev , M.V. Kubrina , O.L. Galkina et all

Keyword(s):

Prenatal Diagnosis ◽

Aortic Arch ◽

Tricuspid Valve ◽

Pulmonary Atresia ◽

Ventricular Septum ◽

Right Atrial ◽

Intact Ventricular Septum ◽

Multicenter Analysis ◽

The Right ◽

Atrial Isomerism

A retrospective analysis of 21 cases of prenatal diagnosed of pulmonary atresia with intact ventricular septum (PA-IVS) is presented. In the study of the four chambers view of the fetal heart the right ventricle was hypoplastic in 16 (76.1 %), normal in 5 (23.8 %) cases. Tricuspid atresia identified in 8 (38 %) cases. The dysplasia of the tricuspid valve identified in 5 (23.8 %) cases. In 2 (8.7 %) cases PA-IVS were in combination with atrioventricular defects. The changes of the tricuspid valve were not identified in 6 (28.5 %) cases. In 100 % cases reduced pulmonary artery with retrograde flow in the pulmonary valve was registrated. Hydrops fetal is detected in 2 (9.5 %) cases. The left aortic arch was in 17 (80.9 %) cases and right aortic arch – 4 (19.1 %). The average gestational age at prenatal diagnosis was 22,6 weeks (range 11–32 weeks). Early diagnosis is represented by one case in 11 weeks. Cardiac defects were registered in 6 (28.5 %) cases and extracardiac defects in 5 (23.8 %) cases. Ventriculocoronary arterial communications were found in 2 (9.5 %) cases of PA-IVS. In 3 (13 %) cases were in combination with heterotaxy syndrome. In 2 (8.7 %) cases PA-IVS were in combination with left atrial isomerism and 1 (4.8 %) with right atrial isomerism. Outcomes in fetuses with PA-IVC: termination of pregnancy — 13 (61.9 %), fetal death — 2 (9.5 %), neonatal death — 5 (23.8 %) cases, only 1 (4.8 %) survivor.

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Prenatal Tricuspid Valve Size as a Predictor of Postnatal Outcome in Patients with Severe Pulmonary Stenosis or Pulmonary Atresia with Intact Ventricular Septum

Fetal Diagnosis and Therapy ◽

10.1159/000357429 ◽

2014 ◽

Vol 35 (2) ◽

pp. 101-107 ◽

Cited By ~ 12

Author(s):

Alexander Lowenthal ◽

Breniel Lemley ◽

Alaina K. Kipps ◽

Michael M. Brook ◽

Anita J. Moon-Grady

Keyword(s):

Tricuspid Valve ◽

Pulmonary Stenosis ◽

Pulmonary Atresia ◽

Ventricular Septum ◽

Intact Ventricular Septum ◽

Valve Size ◽

Postnatal Outcome

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Further morphologic studies on hearts with pulmonary atresia and intact ventricular septum

Cardiology in the Young ◽

10.1017/s1047951100000214 ◽

1991 ◽

Vol 1 (2) ◽

pp. 105-113 ◽

Cited By ~ 31

Author(s):

Robert H. Anderson ◽

Christine Anderson ◽

James R. Zuberbuhler

Keyword(s):

Right Ventricle ◽

Right Ventricular ◽

Tricuspid Valve ◽

Coronary Arteries ◽

Pulmonary Atresia ◽

Outflow Tract ◽

Ventricular Septum ◽

Intact Ventricular Septum ◽

Ventricular Cavity ◽

The Right

SummaryAtresia of the outflow tract of the right ventricle in the presence of an intact ventricular septum poses major problems for surgical management. In the light of known problems, 43 autopsied hearts with this congenital abnormality were evaluated to note the arrangement of the myocardium and the cavity of the right ventricle, the state of the tricuspid valve, the precise substrate for pulmonary atresia, and the presence offistulous communications between the ventricular cavity and the coronary arteries. The specimens could be divided into two groups. The first group (38 hearts) had hypoplasia of the cavity of the right ventricle with mural hypertrophy while the second group (5 hearts) had a dilated right ventricular cavity with thinning of the wall. The larger group could be divided into two subgroups on the basis of the substrate for pulmonary atresia. Twenty hearts had muscular atresia of the right ventricular outflow tract and 18 had a potentially patent outflow tract blocked by an imperforate pulmonary valve. A comparison of these two subgroups revealed that mural hypertrophy and cavitary hypoplasia were more severe in hearts with muscular atresia. The tricuspid valve was hypoplastic and its leaflets were not dysplastic in this group but the leaflets were dysplastic in hearts with valvar pulmonary atresia. Fistulous communications between the ventricular cavity and the coronary arteries were only seen in the cases with muscular atresia. In this series, recognition of muscular pulmonary atresia would have identified those patients with the worst surgical prognosis.

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Pulmonary atresia with intact ventricular septum, right-sided aortic arch and ventriculocoronary connection – prenatal echocardiographic diagnosis

Cardiology in the Young ◽

10.1017/s1047951101000397 ◽

2001 ◽

Vol 11 (3) ◽

pp. 352-354 ◽

Cited By ~ 2

Author(s):

Chandrakant R. Patel ◽

Beverly B. Dahms ◽

Denver Sallee

Keyword(s):

Aortic Arch ◽

Rare Case ◽

Pulmonary Atresia ◽

Ventricular Septum ◽

Intact Ventricular Septum

The authors describe the prenatal echocardiographic diagnosis of a rare case of pulmonary atresia with intact ventricular septum, right-sided aortic arch, and ventriculocoronary connection in a fetus at 21 weeks gestation. The diagnosis was confirmed at autopsy.

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