Atrioventricular Canal Defect, Pulmonary Artery Sling, and Aortic Arch Anomaly in a Patient With Down Syndrome

AbstractWe present two interesting cases of isolated left subclavian artery from the pulmonary artery with symptoms of upper airway obstruction. The first patient had tetralogy of Fallot, pulmonary artery sling, bilateral superior caval veins, and left bronchial isomerism, suggesting heterotaxy syndrome. The second patient had a right aortic arch, isolated left subclavian artery, and bilateral arterial ducts. These two cases are interesting because of their rarity and uncommon presentation.

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SHOULD MANAGEMENT OF COMPLETE ATRIOVENTRICULAR CANAL DEFECT BE INFLUENCED BY COEXISTENT DOWN SYNDROME?

The Lancet ◽

10.1016/s0140-6736(85)92444-4 ◽

1985 ◽

Vol 325 (8438) ◽

pp. 1147-1149 ◽

Cited By ~ 29

Author(s):

Catherine Bull ◽

MichaelL. Rigby ◽

ElliotA. Shinebourne

Keyword(s):

Down Syndrome ◽

Atrioventricular Canal ◽

Atrioventricular Canal Defect ◽

Complete Atrioventricular

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A Massive Pericardial Effusion in a Patient with Common Atrioventricular Canal Defect and Down Syndrome.

Southern Medical Journal ◽

10.1097/00007611-200510001-00115 ◽

2005 ◽

Vol 98 (Supplement) ◽

pp. S43-S44

Author(s):

Michael Nillas ◽

Mahmood Heydarian ◽

Tina Sias

Keyword(s):

Down Syndrome ◽

Pericardial Effusion ◽

Atrioventricular Canal ◽

Atrioventricular Canal Defect

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Agenesis of the left pulmonary artery accompanied by right aortic arch anomaly: CT angiography findings of a case

Vascular Medicine ◽

10.1177/1358863x11427952 ◽

2011 ◽

Vol 17 (2) ◽

pp. 123-124 ◽

Cited By ~ 1

Author(s):

Bilal Battal ◽

Bulent Karaman ◽

Veysel Akgun ◽

Ugur Bozlar ◽

Fatih Ors

Keyword(s):

Pulmonary Artery ◽

Aortic Arch ◽

Ct Angiography ◽

Left Pulmonary Artery ◽

Right Aortic Arch ◽

Aortic Arch Anomaly

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Post-operative course of pulmonary artery pressure after complete atrioventricular canal defect repair

Cardiology in the Young ◽

10.1017/s1047951121002201 ◽

2021 ◽

pp. 1-7

Author(s):

Marie Emeline Pierre Louis ◽

Adnan Bhutta ◽

Adrian Holloway ◽

Peter Gaskin

Keyword(s):

Pulmonary Artery ◽

Right Ventricular ◽

Pulmonary Vascular Resistance ◽

Pulmonary Artery Pressure ◽

Trisomy 21 ◽

Pulmonary Artery Hypertension ◽

Atrioventricular Canal ◽

Atrioventricular Canal Defect ◽

The Right ◽

Complete Atrioventricular

Abstract Complete atrioventricular canal defect is a CHD associated with intra-cardiac shunting of blood, which can lead to irreversible changes in pulmonary vascular resistance and pulmonary artery hypertension if unrepaired. Patients with Trisomy 21 are at risk for early development of pulmonary artery hypertension if left uncorrected.1,2 Objectives: The purpose of this study is to describe the evolution of pulmonary artery hypertension after repair of complete atrioventricular canal defect and to determine the time to normalisation of pulmonary artery pressure in both patients with and without Trisomy 21. Methods: This is a single centre, retrospective analysis of patients with complete atrioventricular canal defect admitted for surgical repair at the University of Maryland Medical Center from 2005 to 2015. T-test or Mann–Whitney test and Chi-Square or Fisher’s exact tests were used to compare the two groups (patients with Trisomy 21 and those without). Repeated measures of analysis of variance and serial measurement analysis were performed. Results: Twenty-nine patients meeting the inclusion criteria underwent repair of complete atrioventricular defects during the study period. The right ventricular pressure estimate remained elevated over time and did not show a significant difference between the two groups. Right ventricular to systolic blood pressure ratios for all patients remained > 0.5 over the time periods assessed. Conclusions: Our study suggests that in patients with complete atrioventricular canal defects, the right ventricular pressure remains elevated and does not normalise on echocardiograms performed up to one year after surgery, suggesting a sustained elevation in pulmonary vascular resistance.

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