Pulmonary atresia with intact ventricular septum and right ventricular dependent coronary circulation through the "vessels of Wearn".

Document Type

Article

Publication Date

7-2013

Identifier

DOI: 10.1016/j.carpath.2012.12.004

Abstract

We present an autopsy case of a male baby born at 35 weeks of gestation with pulmonary atresia with intact ventricular septum (PAIVS), who had coronary blood flow that was dependent on outflow from the right ventricle through the vessels described by Wearn. At 7 weeks of age, he underwent single ventricle palliation consisting of ligation of a patent ductus arteriosus and placement of a modified Blalock-Taussig shunt. The patient experienced a perioperative myocardial infarction, requiring extracorporeal membrane oxygenation. Progressive hemodynamic decline resulted in death 8 days after surgery. Autopsy revealed acute and remote infarctions in both ventricles and fibromuscular dysplasia of the subepicardial and intramural coronary arteries. In 1926, Grant first reported the association between PAIVS and secondary dysplasia of the heart vasculature and hypothesized that the high pressure resulted in dilation of the myocardial sinusoids. Confusion secondary to the unmeritorious dismissal of the myocardial sinusoids has obscured the pathogenesis of PAIVS and led to several publications suggesting second heart field abnormalities as a disease model for PAIVS. We discuss the pathogenesis of PAIVS, the ventriculocoronary arterial connections and the sinusoidal relationship to the vessels described by Wearn, and we attempt to correct the solecism plaguing the nomenclature of myocardial vasculature.

Journal Title

Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology

Volume

22

Issue

4

First Page

298

Last Page

302

MeSH Keywords

Autopsy; Blalock-Taussig Procedure; Coronary Circulation; Coronary Vessels; Ductus Arteriosus, Patent; Extracorporeal Membrane Oxygenation; Fatal Outcome; Fibromuscular Dysplasia; Gestational Age; Heart Defects, Congenital; Hemodynamics; Humans; Infant; Infant, Premature; Ligation; Male; Myocardial Infarction; Palliative Care; Pulmonary Atresia; Treatment Outcome; Ventricular Function, Right

Keywords

FMD; Fibromuscular dysplasia; IVS; LV; PA; PAIVS; PDA; PS; Pulmonary atresia; RV; RVDCC; TV; VCAC; fibromuscular dysplasia; interventricular septum; left ventricle; patent ductus arteriosus; pulmonary atresia with intact ventricular septum; pulmonic atresia; pulmonic stenosis; right ventricle; right ventricular dependent coronary circulation; sIF; secondary intimal fibroplasia; tricuspid valve; ventriculocoronary arterial connection.

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