Ventriculocoronary Artery Connections With the Hypoplastic Left Heart: A 4-year Prospective Study: Incidence, Echocardiographic and Clinical Features

Abstract

Ventriculocoronary connections (VCCs), also called sinusoids, occur with hypoplastic left heart (HLH). Previous reports are limited to case reports, pathologic series, and surgical series with limited detail, which may underestimate the incidence and overestimate the severity of VCCs in HLH. A study was conducted to determine the incidence VCCs in HLH, their effect on survival, and their echocardiographic and clinical features. The echocardiograms and medical records of 100 consecutive neonatal HLH cases were analyzed. All had an aortic and a mitral valve diameter and a left ventricular (LV) volume less than Z-3. For palliation, Norwood, Sano, or hybrid procedures were used, and if the patient was alive, subsequent bidirectional Glenn and extracardiac Fontan procedures were applied. Cases were classified as manifesting mitral and aortic atresia (MAAA), mitral and aortic stenosis (MSAS), or mitral stenosis and aortic atresia (MSAA). All other diagnoses or any case with additional cardiac anomalies were excluded from the study. Overall, VCCs were found in 15% of the cases. They occurred in 56% of the MSAA subtype cases and were not statistically associated with a high mortality rate. However, in one case, large and multiple VCCs definitely caused or contributed to early death. All VCCs had a transmyocardial course, a turbulent color-Doppler flow, and a dominant usually retrograde systolic coronary artery flow pattern. The VCCs were associated (p < 0.05) with MSAA, endocardial fibroelastosis, and ascending aortic size less than 2 mm. As shown by the findings, 15% of the HLH patients had MSAA with VCCs. Unless the VCCs were large or extensive, they did not contribute to mortality. Detailed echocardiographic analysis of VCCs in HLH was feasible. Recent reports emphasize more severe cases.

Beating-Heart Surgery for Hypoplastic Left Heart Syndrome With Coronary Artery Fistulas

“…Vida and colleagues [1] found a 50% failure rate for stage I palliation in patients with this anatomic variant and Sathanandam and colleagues [2] suggested that large VCCs were associated with a poor prognosis. Although the exact mechanism for the increased mortality in this disease subtype remains unclear, some authors have proposed that the VCCs might interfere with adequate myocardial protection during heart decompression on CPB, resulting in ischemia-induced ventricular dysfunction [1,3].…”

“…The ventricular condition could be visually confirmed during the procedure, and a postoperative echocardiogram showed preserved ventricular function. This technique is considered an effective option for minimizing myocardial damage in this patient subtype.(Ann Thorac Surg 2014;98:e103-5) Ó 2014 by The Society of Thoracic Surgeons V entriculocoronary connections (VCCs), also called ventriculocoronary fistulas or sinusoids, are frequently seen in patients with the mitral stenosis-aortic atresia (MS-AA) subtype of hypoplastic left heart syndrome (HLHS) [1,2]. The presence of VCCs is a risk factor for early failure after stage I palliation [1].…”

“…(Ann Thorac Surg 2014;98:e103-5) Ó 2014 by The Society of Thoracic Surgeons V entriculocoronary connections (VCCs), also called ventriculocoronary fistulas or sinusoids, are frequently seen in patients with the mitral stenosis-aortic atresia (MS-AA) subtype of hypoplastic left heart syndrome (HLHS) [1,2]. The presence of VCCs is a risk factor for early failure after stage I palliation [1].…”

Preoperative Stabilization of Infants With Hypoplastic Left Heart Syndrome Before Stage I Palliation

“…Some investigators 10 have reported a diminished survival rate of only 79% for patients with mitral stenosis with aortic atresia compared with the other subtypes for stage I Norwood palliation. Furthermore, recent studies [10][11][12] have indicated that HLHS with mitral stenosis and aortic atresia is associated with ventriculocoronary connections (sinusoids), which adversely affect outcomes and survival. HLHS with an intact or highly restrictive atrial septum is associated with extremely poor outcomes, with a survival rate of only 68% to 70% before discharge from the hospital after the Norwood procedure for patients deemed to be surgical candidates.…”

Coronary Artery Anatomy in Congenital Heart Disease

“…describe in detail the histopathologic characteristics of the ventriculocoronary connections that occur in the setting of ventricular hypoplasia. Historically, these connections have been described by a variety of terms used interchangeably (e.g., coronary artery fistulas, myocardial sinusoidal‐coronary arterial connections, ventriculocoronary connections, and myocardial sinusoids) . While coronary‐cameral fistulas have been encountered in the setting of an isolated atrial septal defect (ASD), VSD, or TOF, it is possible that the VCACs encountered in the setting of a hypertensive and hypoplastic ventricle represent an etiologically, histopathologically, and prognostically discrete subtype of fistulous connections, separate even from myocardial sinusoids …”