Pulmonary Atresia/surgery

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Research Letter

17-Year-Old Man with Pulmonary Atresia and Intact Ventricular Septum Submitted to Fontan Operation, and with Persistent Coronary-Cavitary Fistula

Introduction

Pulmonary atresia with intact ventricular septum is a congenital anomaly with total interruption of blood flow between the right ventricle and the pulmonary trunk, and in general it is not accompanied by associated defects, except for interatrial septal defect, present in 20%, with predominance of patent foramen ovale. Valve atresia results from the lack of embryological development of this fibrous structure, which can be located at the valve level (fibrous membrane) but also at the infundibular level (blind fundus). As a consequence, myocardial hypertrophy and hypoplasia of the right ventricle, hypoplasia of the ring and tricuspid valve, mild tricuspid insufficiency and pulmonary flow dependent on the ductus arteriosus appear. In view of greater hypertension in right ventricle, direct connections with the coronary circulation are formed through sinusoids, with flow towards the aorta. In this situation, it is said that the coronary circulation is dependent on the right ventricle, and when these connections are present in great magnitude, they predispose to myocardial infarction, arrhythmias and right ventricular volume overload, due to retrograde flow from the aorta.

In cases where valve atresia develops later in the fetus, the right ventricular cavity may be well formed with its three portions, the entrance route, the trabecular and the exit route and, as a consequence, there is marked tricuspid insufficiency, even with Ebstein-type alteration of the redundant and myxomatous valve, ventricular wall thinning, ventricular dysfunction and right heart failure, the latter superimposed on hypoxia. In general, it is not accompanied by other associated defects and the pulmonary arteries are of adequate size. In these cases, there is no connection between the right ventricle and the coronary arteries by sinusoids.

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Clinicoradiological Session

Case 3/2014 – 26-year-old Man with Pulmonary Atresia and Ventricular Septal Defect in Late Postoperative Evolution

Edmar Atik

Complementary tests

Electrocardiogram: Showed sinus rhythm, moderate right ventricular (RV) overload without right bundle branch block. Rs morphology in V1 and qRs in V6. Until six years ago, maintained rsr’ morphology in V1. QRS duration was 0.94 from the total correction. AP: +70º, AQRS: +90º, AT: +70º.

Chest radiography: Shows normal cardiac silhouette, rounded shape and normal pulmonary vasculature ().

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Clinicoradiological Session

Case 2/2014 – 20-year-old Woman with Corrected Transposition, Pulmonary Atresia and Aortopulmonary Collateral Arteries

Edmar Atik

Complementary tests

Electrocardiogram () showed junctional rhythm and signs of left ventricular overload on the right. The P wave was negative in II, III, aVF and precordial areas. The QRS complex morphology was RS in V1, rs in V6 and Rs in V6R. The T wave was negative in I, L and more positive in V6R than in V6. AQRS: +160º, AT: 120º, AP: −80º.

Chest radiography shows normal cardiac silhouette (cardiothoracic ratio = 0.45) on the right in situs solitus (gastric bubble on the left) with rounded right ventricular arch. The upper arch on the left was long. Normal pulmonary vasculature and moderate scoliosis ().

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17-Year-Old Man with Pulmonary Atresia and Intact Ventricular Septum Submitted to Fontan Operation, and with Persistent Coronary-Cavitary Fistula

Introduction

Case 3/2014 – 26-year-old Man with Pulmonary Atresia and Ventricular Septal Defect in Late Postoperative Evolution

Complementary tests

Case 2/2014 – 20-year-old Woman with Corrected Transposition, Pulmonary Atresia and Aortopulmonary Collateral Arteries

Complementary tests

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