ABSTRACT
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.
ABSTRACT
Anomalous aortic origin of a coronary artery is a rare congenital anomaly that can cause myocardial ischemia and ventricular arrhythmia. This disease initially manifests as cardiac arrest in half of patients. The indications and surgical strategy remain unclear, especially in patients who are asymptomatic and have poor ischemic findings. We report a surgical strategy to treat anomalous aortic origin of the right coronary artery. A 47-year-old man with a chief complaint of chest tightness was diagnosed with anomalous aortic origin of the right coronary artery, which branches from the left sinus of Valsalva and runs an inter-arterial course. Although no objective myocardial ischemia was identified with blood tests, electrocardiography, or cardiac catheterization, we suspected that the cause of the thoracic symptoms was sharp branching of the right coronary artery, which was compressed between the ascending aorta and the pulmonary artery. To reduce the risk of sudden death in the future, we performed reimplantation of the right coronary artery. Good imaging results were obtained, and the patient is currently undergoing outpatient follow up and has not experienced recurrence of chest symptoms. We conclude that our surgical strategy to treat anomalous aortic origin of a coronary artery may be useful in the clinic.
ABSTRACT
@#Objective To review the experience of the surgical treatment of adult patients with anomalous left coronary artery from the pulmonary artery (ALCAPA). Methods A retrospective, single institution review was conducted on thirty-six adult patients with ALCAPA surgical treatment from November 1991 to November 2017 in Fuwai Hospital. Of these patients, nine were males and twenty-seven were females. The mean age was 36.6±13.3 years. The mean weight was 60.0±9.4 kg. The preoperative echocardiography showed the mean left ventricular ejection fraction (LVEF) was 57%±6% and the mean left ventricular end-diastolic dimension (LVEDD) was 52.3±6.3 mm. Severe mitral regurgitation (MR) was seen in one patient, moderate in five patients. The operative procedures included coronary artery re-implantation in seventeen patients, Takeuchi operation in sixteen patients, ligation of left coronary artery plus coronary artery bypass graft in three patients. In addition, six patients underwent mitral valve repair. Results There was no in-hospital mortality. The mean cardiopulmonary bypass time was 152.5±72.9 min and aortic cross clamp time was 101.9±43.6 min, respectively. The mean mechanical ventilation time and ICU time was 17.3±16.3 h and 43.1±30.7 h, respectively. The mean postoperative LVEF was 59%±6%, which did not significantly improve compared with preoperative LVEF. However, the mean postoperative LVEDD of 46.9±5.9 mm had significant reduction compared with the preoperative LVEDD. Of the six patients with mitral valve repair, one was mild and the other five were trivial. Thirty-five patients (97.2%) completed the follow-up with a mean time of 5.5 years. All the patients survived with New York Heart Association class Ⅰor Ⅱ. Two patients needed interventional occlusion or re-operation due to the fistula of internal tunnel within the pulmonary artery. At the latest echocardiography, the mean LVEF of 69%±7% improved significantly compared with the preoperative LVEF. Mild MR was detected in ten patients, moderate in two patients during the follow-up period. Conclusion The surgical treatment of adult patients with ALCAPA has satisfactory short- and long-term results. The patients who underwent Takeuchi procedure may need re-operation due to fistula of internal tunnel within the pulmonary artery during the long-term follow-up.
ABSTRACT
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.