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

2001 ◽  
Vol 11 (3) ◽  
pp. 352-354 ◽  
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.

Non-confluent pulmonary arteries in a patient with pulmonary atresia and intact ventricular septum: ? a 5th aortic arch with a systemic-to-pulmonary arterial connection

2000 ◽  
Vol 10 (4) ◽  
pp. 419-422 ◽  
Author(s):  
Astolfo Serra ◽  
Francisco Chamie ◽  
R.M. Freedom
Keyword(s):  
Pulmonary Artery ◽  
Aortic Arch ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Left Pulmonary Artery ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Right Pulmonary Artery ◽  
Pulmonary Arterial ◽  
The Right

AbstractMajor abnormalities of pulmonary circulation are uncommon in the patient with pulmonary atresia and intact ventricular septum. Non-confluent pulmonary arteries have only rarely been described in this setting. In this case report, we describe a patient in whom the pulmonary arteries are non-confluent, with the right pulmonary artery supplied through a right-sided arterial duct, and the left pulmonary artery most likely through a fifth aortic arch, thus providing a systemic-to-pulmonary arterial connection. We discuss the various forms of non-confluent pulmonary arteries in the setting of pulmonary atresia and intact ventricular septum.

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

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.

Pulmonary atresia with intact ventricular septum, right-sided aortic arch, and an aorto-pulmonary collateral artery

1999 ◽  
Vol 9 (5) ◽  
pp. 512-515 ◽  
Author(s):  
Chandrakant R. Patel ◽  
Michael L. Spector ◽  
Kenneth G. Zahka
Keyword(s):  
Aortic Arch ◽  
Pulmonary Arteries ◽  
Pulmonary Atresia ◽  
Left Lung ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Descending Aorta ◽  
Left Chest ◽  
Collateral Artery ◽  
The Right

AbstractDescribed is a rare association in a patient with the heart in the left chest, namely pulmonary atresia with intact ventricular septum, fistulous coronary arterial connections, a right-sided aortic arch and an aorto-pulmonary collateral artery feeding one lung. The pulmonary arteries were non-confluent, with the right lung supplied by the right arterial duct originating from the under surface of the right-sided aortic arch, and the left lung supplied through the aorto-pulmonary collateral artery arising from the descending aorta. The surgical management is different in the setting of non-confluent pulmonary arteries.

Survival With Respect to Morphology in Pulmonary Atresia and Intact Ventricular Septum in Sweden

2021 ◽  
Vol 12 (1) ◽  
pp. 27-34
Author(s):  
Stina Manhem ◽  
Katarina Hanséus ◽  
Håkan Berggren ◽  
Britt-Marie Ekman-Joelsson
Keyword(s):  
Pulmonary Atresia ◽  
Early Time ◽  
Ventricular Septum ◽  
Intact Ventricular Septum ◽  
Early Group ◽  
Time Period ◽  
Medical Reports ◽  
One Year ◽  
Survival Risk ◽  
Late Group

Background: Patients born with pulmonary atresia and intact ventricular septum represent a challenge to pediatric cardiologists. Our objective was to study changes in survival with respect to morphology in all children born with pulmonary atresia and intact ventricular septum in Sweden during 36 years. Methods: A retrospective, descriptive study based on medical reports and echocardiographic examinations consisting of those born between 1980 and 1998 (early group) and those born between 1999 and 2016 (late group). Results: The cohort consists of 171 patients (early group, n = 86 and late group, n = 85) yielding an incidence of 4.35 and 4.46 per 100,000 live births, respectively. One-year survival in the early group was 76% compared to 92% in the late group ( P = .0004). For patients with membranous atresia, one-year survival increased from 78% to 98%, and for muscular pulmonary atresia, from 68% to 85%. In patients with muscular pulmonary atresia and ventriculocoronary arterial communications, there was no significant increase in survival. Risk factors for death were being born in the early time period hazard ratio (HR), 6; 95% CI (2.33-14.28) P = .0002, low birth weight HR, 1.26; 95% CI (1.14-1.4) P < .0001 and having muscular pulmonary atresia HR, 3.74; 95% CI (1.71-8.19) P = .0010. Conclusion: The incidence of pulmonary atresia and intact ventricular septum remained unchanged during the study period. Survival has improved, especially for patients with membranous pulmonary atresia, while being born with muscular pulmonary atresia is still a risk factor for death. To further improve survival, greater focus on patients with muscular pulmonary atresia and ventriculocoronary arterial communications is required.

Sign in / Sign up

Export Citation Format

Share Document