Ventriculocoronary artery connections with the hypoplastic left heart: A 4-year prospective study: Incidence, echocardiographic and clinical features

Shyam Sathanandam, Wei Cui, Nguyen Vu Nguyen, Tarek S. Husayni, Andrew H. Van Bergen, Imran Sajan, Chawki El-Zein, Anastasios Charalanpos Polimenakos, Michel N. Ilbawi, David A. Roberson

Research output: Contribution to journalArticle

24 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)1176-1185
Number of pages10
JournalPediatric Cardiology
Volume31
Issue number8
DOIs
StatePublished - Nov 1 2010
Externally publishedYes

Fingerprint

Mitral Valve Stenosis
Arteries
Prospective Studies
Incidence
Endocardial Fibroelastosis
Fontan Procedure
Mortality
Aortic Valve Stenosis
Mitral Valve
Medical Records
Coronary Vessels
Color
Survival

Keywords

  • Hypoplastic left heart syndrome
  • Sinusoids
  • Ventriculocoronary connections

ASJC Scopus subject areas

  • Cardiology and Cardiovascular Medicine
  • Pediatrics, Perinatology, and Child Health

Cite this

Ventriculocoronary artery connections with the hypoplastic left heart : A 4-year prospective study: Incidence, echocardiographic and clinical features. / Sathanandam, Shyam; Cui, Wei; Nguyen, Nguyen Vu; Husayni, Tarek S.; Van Bergen, Andrew H.; Sajan, Imran; El-Zein, Chawki; Polimenakos, Anastasios Charalanpos; Ilbawi, Michel N.; Roberson, David A.

In: Pediatric Cardiology, Vol. 31, No. 8, 01.11.2010, p. 1176-1185.

Research output: Contribution to journalArticle

Sathanandam, S, Cui, W, Nguyen, NV, Husayni, TS, Van Bergen, AH, Sajan, I, El-Zein, C, Polimenakos, AC, Ilbawi, MN & Roberson, DA 2010, 'Ventriculocoronary artery connections with the hypoplastic left heart: A 4-year prospective study: Incidence, echocardiographic and clinical features', Pediatric Cardiology, vol. 31, no. 8, pp. 1176-1185. https://doi.org/10.1007/s00246-010-9783-9
Sathanandam, Shyam ; Cui, Wei ; Nguyen, Nguyen Vu ; Husayni, Tarek S. ; Van Bergen, Andrew H. ; Sajan, Imran ; El-Zein, Chawki ; Polimenakos, Anastasios Charalanpos ; Ilbawi, Michel N. ; Roberson, David A. / Ventriculocoronary artery connections with the hypoplastic left heart : A 4-year prospective study: Incidence, echocardiographic and clinical features. In: Pediatric Cardiology. 2010 ; Vol. 31, No. 8. pp. 1176-1185.
@article{2e27dd74cede4eff87c9f886359d6a5b,
title = "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.",
keywords = "Hypoplastic left heart syndrome, Sinusoids, Ventriculocoronary connections",
author = "Shyam Sathanandam and Wei Cui and Nguyen, {Nguyen Vu} and Husayni, {Tarek S.} and {Van Bergen}, {Andrew H.} and Imran Sajan and Chawki El-Zein and Polimenakos, {Anastasios Charalanpos} and Ilbawi, {Michel N.} and Roberson, {David A.}",
year = "2010",
month = "11",
day = "1",
doi = "10.1007/s00246-010-9783-9",
language = "English (US)",
volume = "31",
pages = "1176--1185",
journal = "Pediatric Cardiology",
issn = "0172-0643",
publisher = "Springer New York",
number = "8",

}

TY - JOUR

T1 - Ventriculocoronary artery connections with the hypoplastic left heart

T2 - A 4-year prospective study: Incidence, echocardiographic and clinical features

AU - Sathanandam, Shyam

AU - Cui, Wei

AU - Nguyen, Nguyen Vu

AU - Husayni, Tarek S.

AU - Van Bergen, Andrew H.

AU - Sajan, Imran

AU - El-Zein, Chawki

AU - Polimenakos, Anastasios Charalanpos

AU - Ilbawi, Michel N.

AU - Roberson, David A.

PY - 2010/11/1

Y1 - 2010/11/1

N2 - 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.

AB - 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.

KW - Hypoplastic left heart syndrome

KW - Sinusoids

KW - Ventriculocoronary connections

UR - http://www.scopus.com/inward/record.url?scp=78449298397&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=78449298397&partnerID=8YFLogxK

U2 - 10.1007/s00246-010-9783-9

DO - 10.1007/s00246-010-9783-9

M3 - Article

C2 - 20820769

AN - SCOPUS:78449298397

VL - 31

SP - 1176

EP - 1185

JO - Pediatric Cardiology

JF - Pediatric Cardiology

SN - 0172-0643

IS - 8

ER -