Measures to Improve In?Hospital Outcomes of Patients Undergoing Surgical Repair for Anomalous Origin of Left Coronary Artery from Pulmonary Artery

Background:Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly leading to progressive left ventricular dysfunction and mitral regurgitation. We conducted this study to investigate various measures to optimize the outcomes of surgical correction for ALCAPA. Materials And Methods: This was a single?centre, retrospective, observational study including consecutive patients operated for ALCAPA. The main outcomes evaluated were in?hospital mortality, duration of mechanical ventilation, and duration of intensive care unit (ICU) stay. Independent sample t? test and Fisher’s exact test were used for the analysis of continuous and categorical variables respectively. Results: 31 patients underwent surgical correction for ALCAPA during the study duration. The median age was 7.3 months with a range of 21 days to 25 months. All patients underwent coronary re?implantation with the coronary button transfer technique. There was no in?hospital mortality, the mean duration of mechanical ventilation and ICU stay was 117.6 hours and 10.7 days respectively. Age at admission, development of acute kidney injury after surgery, lactate levels at 12? and 24?hours post?surgery, and heart rate at ICU admission and 12?hours post?surgery were significantly associated with mechanical ventilation duration longer than 48 hours. Use of a combination of levosimendan and milrinone and elective intermittent nasal continuous positive airway pressure ventilation after extubation in all patients with severe left ventricular dysfunction were helpful in preventing low cardiac output and need for reintubation post?surgery respectively. Conclusion: Surgical correction for ALCAPA by coronary re?implantation has an excellent short?term outcome. Optimal postoperative management is of utmost importance for achieving the best results.

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.

Anomalous left coronary artery from pulmonary artery in an adult

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital cardiac anomaly that can cause myocardial infarction, heart failure and even death in paediatric patients. Only few untreated patients survive till adulthood. Here we present the case of a 28-year-old lady with exertional dyspnoea and chest pain who was diagnosed to have ALCAPA.

Oiigen anomalo de la arteria coronaiia derecha de la arteiia pulmonar en un lactante con soplo cardiaco: A proposito de un caso / Anomalous origin of the right coronary artery from the pulmonary artery in an infant with a heart murmur: Case report

El origen anómalo de la arteria coronaria derecha de la arteria pulmonar es una entidad rara con una incidencia del 0,002 % y potencialmente grave, que puede causar muerte súbita. El diagnóstico frecuentemente es incidental, secundario a evaluaciones por soplos cardíacos a diferencia del origen anómalo de la arteria coronaria izquierda de la arteria pulmonar, reconocido por presentar isquemia miocárdica e insuficiencia cardíaca. Se reporta el caso de una niña de 6 meses evaluada por un soplo y derivada por sospecha de fístula coronaria. La evaluación mediante ecocardiograma doppler color, cateterismo cardíaco y angiotomografía mostró la presencia de una comunicación interauricular ostium secundum pequeña y origen anómalo de la arteria coronaria derecha de la arteria pulmonar. A los 11 meses, se realizó una cirugía de reimplante de la arteria coronaria derecha en la arteria aorta y el cierre de la comunicación interauricular.

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare entity that has an incidence of 0.002 % and can potentially cause sudden death. Unlike to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), usually presented with myocardial ischemia and heart failure; the diagnosis of ARCAPA is often incidental during evaluation of a heart murmur. We report a case of a 6 months old female patient referred to us with a cardiac murmur and a suspicion of coronary fistula. For a proper diagnostic assessment an echocardiogram, a computed tomography angiography and a cardiac catheterization were requested. A small ostium secundum-type atrial septal defect (ASD) and an ARCAPA were revealed. At 11 months old, the patient was scheduled for corrective cardiovascular surgery. The aim of this report is to describe a low incidence and potentially fatal disease.

Anomalous Origin of the Left Circumflex Coronary Artery from the Pulmonary Artery with Atrial Septal Defect / 日本心臓血管外科学会雑誌

<p>Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. This anomaly occurs in approximately one in 300,000 live births. Of the children diagnosed with this syndrome, 90% die within the first year of life because of myocardial ischemia and left ventricular failure. Survival into adulthood is rare and depends upon pre-existing or rapidly developing collateral vessels between the right and left coronary artery. This report concerns the surgical case of anomalous origin of the circumflex coronary artery from the left pulmonary artery complicated with atrial septal defect (ASD). A 34-year-old woman was admitted because of dyspnea on exertion. Echocardiography revealed normal cardiac function with secondary ASD. Coronary catheterization revealed an anomalous origin of the left circumflex coronary artery from the left pulmonary artery. Direct closure of the ASD and coronary artery bypass grafting for the circumflex coronary artery using an internal thoracic artery were performed, and the orifice of the circumflex coronary artery was ligated. The postoperative outcome was excellent. Most of the ALCAPA cases shows the main trunk of the left coronary artery arising from the pulmonary artery. This case demonstrates only the left circumflex coronary artery originating from the left pulmonary artery. Moreover ASD coexisted in this case. To the best of our knowledge, this is a very rare case of its type to be diagnosed and reported.</p>

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.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) in adult age: A 5-case series successfully repaired with surgery / Origen anómalo de la coronaria izquierda en la arteria pulmonar (ALCAPA) en la edad adulta: Una serie de 5 casos reparados con éxito con cirugía

Objective: To describe conduct, evolution and surgical treatment of ALCAPA in adult patients. Material and methods: Series of five cases, > eighteen years old. Clinical data (including surgery and follow-up), and echocardiographic/hemodynamic studies were reviewed. Results: The series comprised four women (80%) and a man (20%), with a mean age of 38.2 (range 18-65) years. Myocardial ischemia was found in three patients (60%), detection of a cardiac murmur in one patient (20%) and aborted sudden death in one patient (20%). Physical examination was normal in every case. Two patients (40%) presented grade II cardiomegaly and venocapillary pulmonary hypertension. In three cases, subendocardial ischemia was found on the EKG without necrosis evidence. Echocardiogram was performed in every patient. On the short axis window turbulent (mosaic) retrograde flow on left interventricular septum and left coronary artery absence was documented. Myocardial scintigraphy was performed on four patients finding moderate to severe anterolateral ischemia. CT angiography was performed on 80% of cases. In 40% of patients, left ventricular end diastolic pressure was elevated in cardiac catheterization. Surgical repair was successful in every case. Coronary reimplantation was performed on two patients, Takeuchi procedure was performed on two patients and revascularization with an internal mammary artery-to-anomalous left coronary artery from the pulmonary artery procedure in one patient. After 48 months of follow-up, every patient is alive in functional class I. Conclusions: Natural history of ALCAPA includes chronic ischemia, ventricular dysfunction and severe arrhythmias that could lead to death. There are very few cases in adults and the likelihood of reaching this age depends on collateral circulation provided by the right coronary artery. This was found in each case in this series, documented by echocardiography and cardiac catheterization. Surgical procedures for this entity include coronary reimplantation, Takeuchi procedure and internal mammary artery-to-anomalous left coronary artery from the pulmonary artery procedure (performed in one patient on this series). Prognosis is good, as long as the congenital defect is corrected.

Objetivo: Describir el comportamiento, evolución y tratamiento quirúrgico de ALCAPA en pacientes adultos. Material y métodos: Serie de cinco casos, mayores de 18 años de edad. Se revisaron los datos clínicos, ecocardiográficos, hemodinámicos, quirúrgicos y del seguimiento. Resultados: La serie comprendió cuatro mujeres (80%) y un varón (20%), con edad promedio de 38.2 años (rango 18-65 años). Se encontró isquemia miocárdica en 60% de los casos, detección de soplo en 20% y muerte súbita abortada en el 20%. La exploración física fue normal en todos los casos. Dos pacientes con cardiomegalia grado II e hipertensión venocapilar. En tres casos el ECG mostró isquemia subendocárdica pero sin evidencia de necrosis. El ecocardiograma reveló flujo turbulento retrógrado en septum interventricular y ausencia de la arteria coronaria izquierda en el eje corto. El gammagrama cardiaco fue positivo para isquemia, moderada a severa en región anterolateral. Se realizó angiotomografía en 80% de los casos. En 40% de los pacientes, la presión diastólica final del ventrículo izquierdo se encontró elevada en el cateterismo cardiaco. La cirugía fue exitosa en todos los casos. La técnica quirúrgica de elección fue el reimplante de la arteria coronaria en dos pacientes, procedimiento de Takeuchi (túnel intrapulmonar) en dos casos y en un solo caso con revascularización con arteria mamaria interna. Después de 48 meses de seguimiento todos los pacientes están vivos y en clase funcional I. Conclusiones: La historia natural de la ALPACA implica isquemia crónica y disfunción ventricular además de arritmias severas que pueden llevar a la muerte. Son pocos los adultos con esta entidad y depende del grado de circulación colateral a partir de la coronaria derecha, esto se encontró en todos nuestros casos y están documentados en el ecocardiograma y confirmados con el cateterismo cardiaco. Las opciones terapéuticas quirúrgicas incluyen el reimplante de la arteria coronaria o bien la revascularización miocárdica con puentes de arteria mamaria. Como ocurrió en una sola de nuestras pacientes que fue la de mayor edad. El pronóstico es bueno, siempre y cuando se corrija la lesión congénita.

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery with a Specific Left Coronary Artery Route / 日本心臓血管外科学会雑誌

We surgically treated a case of anomalous origin of the left coronary artery from the pulmonary artery with the specific route of the left coronary artery in a 17-month-old boy. He had suffered persistent cough and poor weight gain since the age of 4 months. An ultrasound cardiography, at the age of 16 months, revealed retrograde blood flow of the left coronary artery into the main pulmonary artery. Moreover, a chest computed tomography showed an anomalous left coronary artery arising from the bifurcation of the right pulmonary artery and winding in contact on the posterior aortic wall. Though the anomalous left coronary artery shared adventitia with the aortic wall we were able to separate the coronary artery from the aorta, and the patient underwent direct transplantation of the left coronary artery. The postoperative course was uneventful and recovery was rapid.

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.

A Case Report of an Adolescent with Anomalous Origin of the Left Coronary Artery from the Pulmonary Trunk (ALCAPA).

Anomalous origin of the left coronary artery from the pulmonary artery is a serious congenital cardiac defect associated with high mortality rates in infancy. It is undoubtedly a rare defect but its diagnosis during life is possible in most cases, and successful surgery appears within reach. The object of this paper is to report our experience with a fourteen years old girl with this rare anomaly. The presenting symptoms of our patient during infancy were not that are considered classic for this anomaly. Though the child had excessive sweating and symptoms of heart failure during infancy. The girl was suspected to have ALCAPA clinically and echocardiographically finally selective right coronary arteriography established the diagnosis.

Origen anómalo de la coronaria izquierda en la arteria pulmonar: una serie de casos / Anomalous origin of the left coronary artery: a series of cases

La anomalía de la arteria coronaria izquierda que nace en la arteria pulmonar (ALCAPA por su sigla en Inglés: anomalous left coronary artery from the pulmonary artery), es una enfermedad cardiaca congénita, de baja incidencia y de espectro clínico amplio. La principal forma de presentación es la falla cardiaca por miocardiopatía dilatada. Mediante la revisión de historias clínicas se recolectaron cinco casos consecutivos de ALCAPA, de los que se describen sus cuadros clínicos, así como su diagnóstico y tratamiento. Todos fueron dados de alta en mejores condiciones y asisten a controles periódicos. Estos casos ilustran al origen anómalo de la coronaria izquierda, como diagnóstico diferencial de la miocardiopatía dilatada.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a congenital cardiac anomaly with low incidence and a broad clinical spectrum. Its main form of presentation is congestive heart failure due to dilated cardiomyopathy. We reviewed clinical histories and collected 5 consecutive ALCAPA cases; its clinical symptoms, diagnosis and treatment were described. All five patients were discharged in better clinical conditions and continue attending to periodic medical follow-up. These cases illustrate the ALCAPA as part of the differential diagnosis of dilated cardiomyopathy.

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.

Anomalous Origin of The Left Circumflex Coronary Artery from The Left Pulmonary Artery / 대한흉부외과학회지

Anomalous origin of the coronary artery in children is a rare disease. Especially anomalous origin of the left coronary artery from pulmonary artery(ALCAPA) is a fatal condition in neonates with myocardial failure. Among the ALCAPA, origin of the circumflex coronary artery from the pulmonary artery is an extremely rare congenital coronary anomaly. We report a case of reimplantation of the left circumflex coronary artery from the left pulmonary artery to the ascending aorta in a 4-year-old girl.

Severe Tricuspid Insufficiency after Correction of Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery(ALCAPA) / 대한흉부외과학회지

We report a case of an 8 years and 11 month-old male patient who had developed severe tricuspid insufficiency(TI) after correction of anomalous origin of the left coronary artery from the pulmonary artery(ALCAPA). Transthoracic echocardiogram and coronary angiography confirmed ALCAPA, ischemic mitral regurgitation and trivial TI. He underwent direct reimplantation of the left coronary artery to the aortic root by using additional cannulation at the main pulmonary artery for arterial inflow and cardioplegia delivery to the left coronary artery. After the correction of ALCAPA, transesophageal echocardiogram(TEE) revealed good antegrade flow at the aortic implantation site of the left coronary artery and severe TI(Gr III-IV/IV). Cardiopulmonary bypass was reestablished and tricuspid valve was repaired with Kay-type annuloplasty, artificial chordae formation and chordal shortening plasty. The postrepair TEE revealed trivial to mild TI.

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery (ALCAPA) with Intramural Aortic Route / 日本心臓血管外科学会雑誌

We report a 5-year-old girl with a diagnosis of an anomalous origin of the left coronary artery from the pulmonary artery with an intramural aortic route. The left coronary artery entered the aortic wall running parallel to the aorta. With the aid of cardiopulmonary bypass, she underwent establishment of two coronary artery systems by intraaortic reconstruction (unroofing and anastomosis). Her postoperative course was uneventful. Postoperative cineangiogram demonstrated patency and prograde flow in the new coronary systems.

Anomalous Origin of Left Coronary Artery from Pulmonary Artery:Report of an Adult Case

Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is a rare congenital cardiovascular anomaly. The mortality rate among infants and children without operation has been eighty to ninety-five percents and few patients survive till teen-age or adulthood. This anomaly was detected during elective coronary angiogram in a 32 year-old female patient with atypical chest pain. Reversible ischemia was demonstrated on myocardial 201Tl-SPECT. Coronary angiogram revealed anomalous origin of left coronary artery from pulmonary artery.