Case Report: ALCAPA syndrome: successful repair with an anatomical and physiological alternative surgical technique

Mollinedo, Luis Gustavo Vilá; Uribe, Andrés Jaime; Chimal, José Luis Aceves; Martínez-Rubio, Roberto Pablo; Hernández-Romero, Karen Patricia

doi:10.12688/f1000research.8823.1

Cited by 6 publications

(4 citation statements)

References 13 publications

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“…Without early and accurate diagnosis and treatment, the natural course of the disease is devastating and 90% untreated children die in the first year of life because of massive myocardial injury and following end-stage heart failure. [3,4] With appropriate diagnosis and emergency cardiac surgery, they have a much better chance to survive. However, owing to the lack of specificity in the clinical manifestations, ALCAPA has a higher rate of false initial diagnosis.…”

Section: Introductionmentioning

confidence: 99%

Misdiagnosed anomalous left coronary artery from the pulmonary artery as endocardial fibroelastosis in infancy

et al. 2017

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Introduction:Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare but severe congenital cardiac malformation. The prognosis mainly depends on the early and accurate diagnosis and treatment. However, without a typical and specific clinical manifestation in early stage, ALCAPA has a higher rate of false initial diagnosis.Diagnostic and therapeutic procedure:Three infants with impaired left ventricle (LV) function, LV enlargement, mitral valve regurgitation (MR), and LV endocardium thickness were initially diagnosed as endocardial fibroelastosis (EFE). Due to the treatment effectiveness with prednisone acetate and digoxin, abnormal Q waves with T inversion, and dilated right coronary artery (RCA), the diagnosis of ALCAPA was suspected. Lastly, cardiac angiography confirmed the diagnosis. All of them were transferred to the cardiac surgery department and received a successful surgical repair. The follow-up results showed that abnormal Q waves with T waves inversion on electrocardiogram gradually regressed and disappeared, LV ejection fraction and LV dilation returned to a normal range after surgery, with alleviation of MR. Besides, endocardial thickness secondary to ischemia also returned to normal.Conclusion:ALCAPA should be suspected when confronted with patients with left heart enlargement, impaired left ventricular function, and signs of myocardial ischemia, particularly in infancy. EFE is an important differential diagnosis and may also arise as a result of ALCAPA. Abnormal Q waves with T waves inversion, particularly in avL, dilated RCA and increased ratio of RCA/AO are important differential key points for the identification of ALCAPA and EFE. Awareness of this condition is essential for prompt recognition and referral to a tertiary cardiac center to enable early surgical intervention and improved prognosis for these children.

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Section: Introductionmentioning

confidence: 99%

Misdiagnosed anomalous left coronary artery from the pulmonary artery as endocardial fibroelastosis in infancy

et al. 2017

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“…After identification of all patients with ACAPA who underwent repair from 2007 to 2016, demographics, preoperative and intraoperative variables, and postoperative complications were compared between survivors and nonsurvivors. The primary outcomes included (1) in-hospital mortality and (2) the need for postoperative extracorporeal membrane oxygenation (ECMO) support. Multivariable logistic regression was used to determine preoperative and intraoperative risk factors for these outcomes.…”

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confidence: 99%

“…Papillary muscle damage and changes in mitral annular dimensions from left ventricular dilation can result in mitral regurgitation. 1 Less commonly, ALCAPA may manifest later in life, presumably in patients in whom sufficient intercoronary collateral circulation develops. ARCAPA, conversely, affects about 0.002% of the population and typically manifests beyond infancy.…”

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Factors Associated With Adverse Outcomes After Repair of Anomalous Coronary From Pulmonary Artery

Straka

Allan

Jacobs

et al. 2019

The Annals of Thoracic Surgery

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“…There are various surgical interventions to repair ALCAPA and most are deemed successful with the individuals returning toward baseline post-surgery. Such surgical interventions include direct reimplantation of the LCA, the Takeuchi (transpulmonary baffling) repair, ligation of the LCA, the bay window technique and autologous pulmonary wall flap technique 1,9,11,12 . Patients who present with mitral regurgitation usually have improvement of their mitral regurgitation following surgery even though it is not a direct focus of the intervention 11,13 .…”

Section: Introductionmentioning

confidence: 99%

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2018

J Pediatrics & Pediatr Med

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Anomalous left coronary artery to pulmonary artery (ALCAPA) is a congenital heart defect in which the left coronary artery (LCA) originates from the pulmonary trunk instead of from the aorta. This disease occurs in 1 in 300,000 births and, if untreated, 90% of these neonates die within the first year. Individuals who live beyond this tend to be asymptomatic and either experience sudden death at an average age of 35 or present with cardiac abnormalities, including myocardial ischemia, arrhythmia, or mitral regurgitation. There are various surgical interventions used to treat ALCAPA, and the establishment of a two coronary artery system is the preferred treatment of choice. We report a case of a five-year-old presenting with supraventricular tachycardia since birth who, upon diagnosis of ALCAPA by angiography, was treated by surgical ligation. Thirty years later, he returned to a cardiologist with symptoms of congestive heart failure. The initial presentation of this patient is unusual because the manifestation of ALCAPA usually occurs within the first few months of birth and because ligation is no longer the preferred method of intervention. We discuss this unique case, suggest possible associations between ALCAPA and arrhythmias, review the various surgical methods used to treat ALCAPA, and evaluate the long-term outcome of ligation.

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Case Report: ALCAPA syndrome: successful repair with an anatomical and physiological alternative surgical technique

Cited by 6 publications

References 13 publications

Misdiagnosed anomalous left coronary artery from the pulmonary artery as endocardial fibroelastosis in infancy

Misdiagnosed anomalous left coronary artery from the pulmonary artery as endocardial fibroelastosis in infancy

Factors Associated With Adverse Outcomes After Repair of Anomalous Coronary From Pulmonary Artery

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