Descripción de dos casos de síndrome de ALCAPA / Description of two cases of ALCAPA syndrome

Resumen El origen anómalo de la arteria coronaria izquierda desde la arteria pulmonar o, por su sigla en inglés, síndrome de ALCAPA (Anomalous origin of the Left Coronary Artery from the Pulmonary Artery), también conocido como síndrome Bland-White-Garland es una rara cardiopatía congénita; sin embargo, es una de las causas más comunes de falla cardiaca, isquemia e infarto de miocardio en niños, que, en ausencia de tratamiento, alcanza una tasa de mortalidad del 35 al 85 % en el primer año de vida. Hay dos tipos de síndrome de ALCAPA, aquel que se presenta en infantes y el que ocurre en adultos. El segundo tipo es raro y puede manifestarse como infarto de miocardio, disfunción ventricular izquierda e insuficiencia mitral, o isquemia silente que podría llevar a muerte súbita. Se presenta el caso de una paciente de 30 años, con cardiopatía dilatada y disfunción ventricular severa, secundaria a síndrome de ALCAPA, y el caso de un paciente de 61 años con cuadro de dolor torácico en quien se documentó el mismo síndrome.

Abstract The anomalous origin of the left coronary artery from the pulmonary artery or its acronym ALCAPA syndrome (Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery), also known as Blande-Whitee-Garland syndrome, is a rare congenital heart disease. However, one of the most common causes of heart failure, ischemia and myocardial infarction in children, which in the absence of treatment, reaches a mortality rate of 35 to 85% in the first year of life. There are two types of ALCAPA syndrome, that occurs in infants and adults. The presentation of the second type is rare and can manifest as myocardial infarction, left ventricular dysfunction and mitral insufficiency, or silent ischemia that could lead to sudden death. We present the case of a 30-year-old patient with dilated cardiomyopathy and severe ventricular dysfunction, secondary to the ALCAPA syndrome, another case of a 61-year-old patient with chest pain where the mentioned syndrome was documented.

Nacimiento anómalo de la arteria coronaria izquierda desde la arteria pulmonar, síndrome de ALCAPA. Primer reporte de caso en Uruguay / Anomalous origin of the left coronary artery from the pulmonary artery, ALCAPA syndrome. First case report in Uruguay / Nascimento anômalo da artéria coronária esquerda a partir da artéria pulmonar, síndrome de ALCAPA. Relato do primeiro caso no Uruguai

Resumen: El nacimiento anómalo de la arteria coronaria izquierda desde la arteria pulmonar (síndrome de ALCAPA) es una rara entidad con una mortalidad de 90% durante el primer año de vida. Este hecho hace que su presencia en el adulto sea excepcional. Presentamos el primer caso reportado en nuestro país en un adulto. Se trata de una paciente de 32 años en estudio por historia de ángor de esfuerzo en la que el ecocardiograma brindó elementos sugestivos de esta entidad que se confirmó con coronariografía. Se intervino quirúrgicamente de forma exitosa con técnica de Takeuchi.

Summary: The anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) is a rare entity with a high infant mortality rate during first year of life reaching up to 90%. This fact makes this condition extremely uncommon among adults. We report for the first time in our country an adult presentation in a 32-year-old woman. This patient presented with a history of exertional angina and a transthoracic echocardiogram showed suggestive clues for the diagnosis of ALCAPA, then confirmed with coronary angiography. The patient underwent successfull surgical correction with Takeuchi technique.

Resumo: O nascimento anômalo da artéria coronária esquerda a partir da artéria pulmonar (síndrome de ALCAPA) é uma entidade rara com uma mortalidade de 90% durante o primeiro ano de vida. Este fato faz com que sua apresentação da idade adulta seja excepcional. Apresentamos o primeiro caso reportado dessa síndrome no nosso país. Se trata de uma paciente de 32 anos em estudo por uma historia de angina de esforço na qual o ecocardiograma brindou elementos sugestivos dessa entidade sendo confirmada posteriormente com angiografia coronaria. Se realizou uma intervenção cirúrgica exitosa com a técnica de Takeuchi.

Late presentation of ALCAPA syndrome in an elderly Asian lady

Coronary artery anomalies are often discovered incidentally during cardiac catheterization or computed tomography coronary angiography and may involve the affected coronary artery origin and its course. Coronary artery anomalies are associated with congenital heart disease. The affected coronary arteries may have an unusual high take off origin, origin from contralateral or non-coronary sinus, origin from the pulmonary artery, single coronary system or coronary artery fistula.

A 69-year-old Woman with Anomalous Origin of Left Coronary Artery from the Pulmonary Artery: Surgical Repair Using a Trap-door Flap

We present a patient with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), which was diagnosed and corrected in her 60 s. The patient is the oldest documented survivor of ALCAPA who underwent a surgical repair. ALCAPA should be corrected surgically to restore the dual coronary system at any age and this case shows that the surgical procedure may be performed safely even in an elderly patient.

Early Result of Surgical Management of the Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery / 대한흉부외과학회지

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly, but is one of the most common causes of myocardial ischemia which would result in high mortality within the first year of life. This is our early result of the surgical management for these patients. MATERIAL AND METHOD: From June 1989 to July 2003, 6 patients with ALCAPA and one patient with ARCAPA (Anomalous origin of the Right coronary artery from the pulmonary artery) underwent surgical repair. We have reviewed the all medical records, electrocardiogram, chest X-ray and echocardiography retrospectively. RESULT: Three of the patients were boys and four were girls. The median age at the operation was 5.4 months (Range: 3~33 months). The average body weight of at the operation was 6.7 kg (Range: 3.7~11.3 kg). A mean follow up period was 18 months. Only 3 patients were initially diagnosed as ALCAPA. And 3 patients had moderate mitral regurgitation. Immediate coronary artery reimplantation on diagnosis with the aim of restoring a two-coronary system circulation was done. The average bypass time was 114+/-37 minutes, and the average aortic cross clamping time was 55+/-22 minutes. The average stay of intensive care unit was 5+/-3 days, the mean mechanical ventilator time was 38+/-45 hours and the hospital stay after operation was 12+/-5 days. There were significant improvements in electrocardiogram and chest X-ray of the all patients except one late death patient. The ventricular function showed almost normal recovery after operation; the EF (Ejection Fraction) increased from 41.2+/-10.3% to 60.5+/-15.8% within 1 month and to 59.8+/-13.9% within 1 year after operation, the SF (Shortening Fraction) increased from 23.6+/-4.7% to 38.6+/-8.4% within 1 month and to 37.4+/-7.9% within 1 year after operation, LVEDDI (Left Ventricular End-diastolic Dimension Index) decreased from 100.8+/-25.6 mm/m2 to 90.3+/-19.2 mm/m2 within 1 month and to 79.3+/-15.8 mm/m2 within 1 year after operation. Concomitant mitral repair was done in two patients with anterior mitral leaflet prolapse. In every patient, mitral valve showed less than mild regurgitation during follow up. One late death occurred in which patient Dor procedure was applied 10 months after initial operation due to the dilated cardiomyopathy. CONCLUSION: In the management of this rare and could be fatal Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), early suspicion and correct diagnosis is of most important.But, after diagnosis, immediate restoration of 2 coronary systems could result in good outcome.

Surgical Treatment of Anomalous Origin of Coronary Artery from the Pulmonary Artery: Postoperative Changes of Ventricular Dimensions and Mitral Regurgitation / 대한흉부외과학회지

BACKGROUND: The aims of this study are to verify the result of the surgical treatment of ALCAPA and to identify the postoperative changes of left ventricular dimensions and mitral regurgitation (MR). MATERIAL AND METHOD: Fifteen patients operated on since 1985 were included in the study. The patients operated on before 1998 (n=9) showed heterogeneous properties with various surgical strategies and cardiopulmonary bypass techniques. However, six patients were operated on with the established surgical strategy since 1998; 1) Dual perfusion and dual cardioplegic solution delivery through ascending aorta and main pulmonary artery, 2) Coronary transfer by rolled-conduit made of pulmonary artery wall flap, and 3) Additional mitral valvular procedure was not performed. RESULT: Median age of the study group was 6 months (1 month to 34 years). The operative methods were left subclavian artery to left coronary artery anastomosis in 1, simple ligation in 2, Takeuchi operation in 2, and coronary reimplantation in 10 pateints. The mean follow up period was 5.5<5.8 years (2 months 14 years). There were one early death (6.7%) and one late death. Overall 5-year survival rate was 85.6+/-9.6%. The Z-value of left ventricular end-diastolic and end-systolic dimensions were 6.4+/-3.0 and 5.1+/-3.6 preoperatively, and decreased to 1.7+/-1.9 and 0.8+/-1.6 in 3 months (p<0.05). Significant preoperative MR was identified in 6 patients (40%) and all the patients showed immediate improvement of MR within 1 month postoperatively. There were 3 cases of reoperation due to coronary anastomosis site stenosis and recurrence of MR. However, there was no mortality nor late reoperation in the patients operated on after 1998. CONCLUSION: The surgical treatment of ALCAPA showed favorable survival and early recovery of ventricular dimensions and mitral valvular function. Although long-term reintervention was required in some cases of earlier period, all the cases after 1998 showed excellent surgical outcome without long-term problem.