Echocardiographic Diagnosis, Surgical Treatment, and Outcomes of Anomalous Left Coronary Artery from the Pulmonary Artery

Shivani G. Patel, Michele A. Frommelt, Peter C. Frommelt, Shelby Kutty, Jonathan W Cramer

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14 Citations (Scopus)

Abstract

Background Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare condition that can be difficult to diagnose by echocardiography alone. The purpose of this study was to describe the clinical and echocardiographic presentation of ALCAPA, create a set of critical echocardiographic diagnostic criteria, and report outcomes. Methods A retrospective review was conducted of all patients diagnosed with isolated ALCAPA at two major cardiac centers between 1990 and 2015. Results Of the 37 patients identified, only 54% presented in infancy. The anomalous coronary artery (CA) origin was clearly imaged in only 54% of echocardiographic examinations. However, other consistently identified echocardiographic markers were found, including left CA flow reversal (91%), collateral CA flow (85%), right CA dilation (81%), abnormal pulmonary artery flow (79%), mitral regurgitation (74%), left ventricular dysfunction (66%) and endocardial fibroelastosis (57%). Presenting echocardiograms had five of seven markers in 85% of patients. Left ventricular dysfunction was the most common marker in infants (89% vs 38%, P =.005); older children were more likely to have collateral formation visualized by color Doppler (100% vs 75%, P =.04). Following surgery, there were no early surgical deaths. The median follow-up duration was 10.3 years. At last follow-up, 92% had normal left ventricular function, 3% had moderate or worse mitral regurgitation, and 17% had required reintervention. Conclusions Echocardiographic markers can reliably identify ALCAPA; these markers vary with the age of presentation. Surgical outcomes are excellent, and most patients will recover left ventricular and mitral valve function.

Original languageEnglish (US)
Pages (from-to)896-903
Number of pages8
JournalJournal of the American Society of Echocardiography
Volume30
Issue number9
DOIs
StatePublished - Sep 2017

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Pulmonary Artery
Coronary Vessels
Mitral Valve Insufficiency
Left Ventricular Dysfunction
Endocardial Fibroelastosis
Mitral Valve
Left Ventricular Function
Echocardiography
Dilatation
Color

Keywords

  • ALCAPA
  • Diagnosis
  • Echocardiography
  • Outcomes

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging
  • Cardiology and Cardiovascular Medicine

Cite this

Echocardiographic Diagnosis, Surgical Treatment, and Outcomes of Anomalous Left Coronary Artery from the Pulmonary Artery. / Patel, Shivani G.; Frommelt, Michele A.; Frommelt, Peter C.; Kutty, Shelby; Cramer, Jonathan W.

In: Journal of the American Society of Echocardiography, Vol. 30, No. 9, 09.2017, p. 896-903.

Research output: Contribution to journalArticle

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abstract = "Background Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare condition that can be difficult to diagnose by echocardiography alone. The purpose of this study was to describe the clinical and echocardiographic presentation of ALCAPA, create a set of critical echocardiographic diagnostic criteria, and report outcomes. Methods A retrospective review was conducted of all patients diagnosed with isolated ALCAPA at two major cardiac centers between 1990 and 2015. Results Of the 37 patients identified, only 54{\%} presented in infancy. The anomalous coronary artery (CA) origin was clearly imaged in only 54{\%} of echocardiographic examinations. However, other consistently identified echocardiographic markers were found, including left CA flow reversal (91{\%}), collateral CA flow (85{\%}), right CA dilation (81{\%}), abnormal pulmonary artery flow (79{\%}), mitral regurgitation (74{\%}), left ventricular dysfunction (66{\%}) and endocardial fibroelastosis (57{\%}). Presenting echocardiograms had five of seven markers in 85{\%} of patients. Left ventricular dysfunction was the most common marker in infants (89{\%} vs 38{\%}, P =.005); older children were more likely to have collateral formation visualized by color Doppler (100{\%} vs 75{\%}, P =.04). Following surgery, there were no early surgical deaths. The median follow-up duration was 10.3 years. At last follow-up, 92{\%} had normal left ventricular function, 3{\%} had moderate or worse mitral regurgitation, and 17{\%} had required reintervention. Conclusions Echocardiographic markers can reliably identify ALCAPA; these markers vary with the age of presentation. Surgical outcomes are excellent, and most patients will recover left ventricular and mitral valve function.",
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N2 - Background Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare condition that can be difficult to diagnose by echocardiography alone. The purpose of this study was to describe the clinical and echocardiographic presentation of ALCAPA, create a set of critical echocardiographic diagnostic criteria, and report outcomes. Methods A retrospective review was conducted of all patients diagnosed with isolated ALCAPA at two major cardiac centers between 1990 and 2015. Results Of the 37 patients identified, only 54% presented in infancy. The anomalous coronary artery (CA) origin was clearly imaged in only 54% of echocardiographic examinations. However, other consistently identified echocardiographic markers were found, including left CA flow reversal (91%), collateral CA flow (85%), right CA dilation (81%), abnormal pulmonary artery flow (79%), mitral regurgitation (74%), left ventricular dysfunction (66%) and endocardial fibroelastosis (57%). Presenting echocardiograms had five of seven markers in 85% of patients. Left ventricular dysfunction was the most common marker in infants (89% vs 38%, P =.005); older children were more likely to have collateral formation visualized by color Doppler (100% vs 75%, P =.04). Following surgery, there were no early surgical deaths. The median follow-up duration was 10.3 years. At last follow-up, 92% had normal left ventricular function, 3% had moderate or worse mitral regurgitation, and 17% had required reintervention. Conclusions Echocardiographic markers can reliably identify ALCAPA; these markers vary with the age of presentation. Surgical outcomes are excellent, and most patients will recover left ventricular and mitral valve function.

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