[First diagnosis of ALCAPA syndrome in adulthood: a rare cause of cardiac arrest]. / Erstdiagnose ALCAPA-Syndrom im Erwachsenenalter ­ seltene Ursache eines Herz-Kreislauf-Stillstands.

A 42-year-old patient with return of spontaneous circulation (ROSC) following an out-of-hospital cardiac arrest was referred to the authors' emergency department. The initial rhythm was ventricular fibrillation. A computed tomography scan and subsequent coronary angiography revealed anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome as the cause of this condition. A thickened right coronary artery with significant collateral blood flow to the left coronary artery was observed. After initial treatment in the authors' intensive care unit, surgical intervention was performed. The patient was discharged from hospital without any neurological damage.

Anomalous origin of left coronary artery from the pulmonary artery: Our 30 years of surgical experience and outcomes.

Background: The aim of this study was to evaluate our 30 years of surgical experience and outcomes in management of anomalous origin of left coronary artery from the pulmonary artery with various age groups. Methods: Between March 1992 and August 2022, a total of 21 patients (10 males, 11 females; mean age: 16.3±15.9 years; range, 1 to 64 years) diagnosed with anomalous origin of left coronary artery from the pulmonary artery who underwent successful surgical repair were retrospectively analyzed. An initial diagnosis was made with two-dimensional echocardiography. Cardiac catheterization and angiography were performed in all our patients. Results: The median follow-up was five (range, 1 to 14) years. The mean left ventricular ejection fraction was 43.47±14.30% with associated moderate-to-severe mitral regurgitation in seven (33.33%) patients. Coronary button transfer was performed in 15 (71.42%) patients, Takeuchi repair in four (19.04%) patients, and ligation of anomalous left main coronary artery and ligation with great saphenous venous graft in one (4.76%) patient. There was no mortality. However, two (9.52%) patients had prolonged intensive care unit stay (>7 days). At the final follow-up, the mean left ventricular ejection fraction improved to 57.47±4.97%. Regression of moderate mitral regurgitation was observed in four (66.6%) patients. Conclusion: Preoperative left ventricular function is a major risk factor of perioperative mortality and morbidity. Mitral valve intervention is not warranted concomitantly in patients with moderate mitral regurgitation, if there are no structural lesions. Early diagnosis and meticulous surgical technique can yield excellent results and good long-term outcomes.

Anomalous origin of the left coronary artery from the right pulmonary artery.

ALCAPA should be considered in the differential diagnosis of myocarditis, and contrast-enhanced CT or catheterization should be considered even if coronary artery abnormalities are not detected on echocardiography.

ALCAPA in adult asymptomatic patient: A case report.

INTRODUCTION: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital heart disease that usually presents with heart failure symptoms in infants. Without surgical correction, the condition has a high infant mortality rate. However, patients with ALCAPA can remain asymptomatic for decades in some cases, and the risk of sudden death decreases in adulthood. PRESENTATION OF CASE: We present the case of a 52-year-old female who was incidentally diagnosed with ALCAPA during a routine medical evaluation. As the patient age, was asymptomatic, had good coronary collateral circulation, a medical treatment strategy was chosen and the patient was discharged in a good physical condition. And during the three-month follow-up, no cardiovascular complications were observed. DISCUSSION: The appearance and severity of symptoms in patients with ALCAPA can vary depending on factors such as the closure of the patent ductus arteriosus (PDA), pressure gradient between arteries, collateral development, and coronary anatomy. Surgical intervention is typically recommended, but in select cases such us, conservative management may be considered for elderly patients due to increased surgical risks and potentially lower risk of sudden cardiac death. Individualized patient assessment is crucial in determining the optimal treatment strategy for ALCAPA, considering the available evidence and limitations. CONCLUSION: The management of asymptomatic patients with ALCAPA remains a subject of discussion, and further research is needed to standardize the clinical approach for this subgroup of patients and to compare survival rates between surgical correction and medical therapy.

Surgical Reconstruction of the Anomalous Left Coronary Artery From the Pulmonary Artery.

The anomalous left coronary artery from the pulmonary artery is a rare congenital disease. Early surgical reconstruction is mandatory to prevent adverse cardiac events. Direct coronary button transfer, vein graft interposition, ligation, and coronary artery bypass construction are the most commonly used techniques. This case report presents a modified technique of Dacron graft interposition and reimplantation anomalous left coronary artery from the pulmonary artery on the ascending aorta.

Anomalous coronary artery from the pulmonary artery diagnosed in adulthood: a case series on variations of coronary anatomy and the diagnostic value of cardiac magnetic resonance imaging.

Background: Anomalous coronary artery from the pulmonary artery is a rare congenital disorder with high mortality rates in infancy. Adult cases can present with life-threatening arrhythmias and sudden cardiac death. Case summary: We report three cases of adults with anomalous pulmonary origin of each of the main coronary branches. The first patient with an anomalous left coronary artery from the pulmonary artery presented with an out-of-hospital cardiac arrest. The second patient with an anomalous circumflex artery from the pulmonary artery was evaluated for a bicuspid aortic valve and a suspected coronary fistula but was otherwise asymptomatic. The third patient with an anomalous right coronary artery from the pulmonary artery presented with anginal symptoms. In all cases, the diagnosis was made by cardiac computed tomography or coronary angiography. Cardiac magnetic resonance imaging was performed in all patients to guide clinical decision making on surgical or non-invasive management. All patients underwent surgical repair. In two patients, a dual coronary artery system was restored by aortic reimplantation of the anomalous coronary artery. In one patient, aortic reimplantation was unsuccessful due to poor vessel quality and the anomalous coronary artery was ligated. Clinical follow-up during 1.8-9.7 years did not show any cardiovascular complications and all patients are currently alive and asymptomatic. Discussion: Anomalous coronary artery from the pulmonary artery can have various clinical presentations in adulthood. Cardiac magnetic resonance imaging is a useful modality to guide selection of patients who might have symptomatic or prognostic benefit from surgical repair.

The salutary effect of peritoneal dialysis catheters on enhanced recovery among high-risk pediatric patients undergoing the left coronary transfer procedure: a cohort study.

BACKGROUND: Evidence for peritoneal dialysis catheter (PDC) usage in pediatric patients undergoing surgery for deteriorating cardiac dysfunction is lacking. This investigation explored factors associated with PDC usage and its effectiveness in children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). METHODS: Eighty-four children undergoing left coronary artery transfer were retrospectively recruited. The primary endpoint was the postoperative ratio of the general ward/[intensive care unit (ICU)] length of stay. Univariable and multivariable analyses were fitted to assess factors related most strongly to PDC and the ratio of general ward/ICU length of stay. RESULTS: Of the 84 patients, 17 (20.2%) underwent postoperative PDC placement. Patients with extreme cardiac dysfunction [left ventricular ejection fraction (LVEF) ≤25%] were much more likely to require a PDC (OR, 9.88; 95% CI, 2.13-45.76; P = 0.003). Moreover, univariate analysis indicated that concomitant mitral repair significantly decreased the likelihood of PDC placement (OR, 0.25; 95% CI, 0.07-0.85; P = 0.026). In those with cardiac dysfunction (LVEF ≤50%), PDC use was associated with a reduced ratio of ward/ICU length of stay (B, - 1.62; 95% CI, - 2.77- -0.46; P = 0.008), as was age ≤ 12 months (B, - 1.57; 95% CI, - 2.88- -0.26; P = 0.02). At the 1-year follow-up, cardiac improvement was significantly greater in patients with PDC usage than in those without it (P <  0.001), and the number of mitral recoveries was comparable between the groups (64.2% vs. 53.3%, P = 0.434). CONCLUSION: In cohorts with ALCAPA, PDC placement following surgery may be necessary for patients with extreme cardiac compromise, while concomitant mitral repair can probably reduce their usage rate. PDC is beneficial in conferring an improvement in cardiac and mitral performance. Importantly, after patients are transferred from the ICU, recovery efficiency in the general ward can be enhanced by PDC placement, and hospital discharge can therefore be achieved early, especially for patients younger than 12 months or with LVEF ≤50%.

Long-term surgical results of anomalous origin of the left coronary artery from the pulmonary artery repair in infants and older patients.

OBJECTIVES: The objective of this study was to compare the long-term surgical results of anomalous origin of the left coronary artery (LCA) from the pulmonary artery repair between infants and older patients. METHODS: This is a retrospective study of patients undergoing anomalous origin of the LCA from the pulmonary artery repair between 1982 and 2018. RESULTS: Thirty-five patients were enrolled and divided into two groups based on their age: infants (<1 year of age: n = 11) and older patients (>1 year of age: n = 24). Median age was 5.2 months in infants and 6.3 years in older patients. The procedure included 24 LCA implantations and 11 intrapulmonary baffle repairs. Early mortality was recorded only in two infants (18%). Early complications included two low output syndrome (18%). Survival and freedom from reoperation at median follow-up of 17.2 years were not significantly different between groups (survival, 81.8 ± 11.6% vs. 94.1 ± 5.7% [p = .3846]; freedom from reoperation, 77.8 ± 13.9% vs. 90.0 ± 6.8% [p = .1175]). Mitral regurgitation (MR) developed substantially in two older patients in the long-term. In infants, significant improvements of fractional shortening (p = .0252) and left ventricular (LV) end-systolic (p = .0142) and end-diastolic diameter (p = .0157) occurred at 1 month. CONCLUSIONS: Although there was no significant difference in survival and reoperation rate, the postoperative LV remodeling occurred more evident and rapid in infants. Worsening of MR in the long-term was observed only in older patients.

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.

Origen anómalo de la arteria coronaria izquierda a partir de la arteria pulmonar / Anomalous origin of left coronary artery from the pulmonary artery

Introducción: El origen anómalo de la arteria coronaria izquierda que nace en la arteria pulmonar, conocido como síndrome de Bland, White y Garland, es una enfermedad cardiaca congénita, de baja incidencia, espectro clínico variable y no es exclusiva de la edad pediátrica. Tiene una alta mortalidad en el primer año de vida si no se diagnostica y trata oportunamente. Objetivo: Describir las características del diagnóstico del síndrome de Bland, White y Garland. Métodos: Se realizó la caracterización de la entidad en Cuba en un decenio (2005 a 2015). Se incluyeron todos los pacientes con manifestaciones clínicas sugerentes de síndrome de Bland, White y Garland y confirmación ecocardiográfica. Se realizó el cálculo de la prevalencia de la entidad y se empleó el porcentaje en las variables cualitativas y la prueba exacta de Fisher. La validación estadística de los resultados de la investigación adoptó nivel de significación menor a 5 por ciento. Resultados: La prevalencia estimada en esa década en Cuba estaba alrededor de 2,9 por 300 000 nacidos vivos. Se demuestra la variabilidad en sus manifestaciones clínicas y amplio rango de edad de presentación. El tratamiento es quirúrgico con excelente supervivencia y pronóstico. Conclusiones: El diagnóstico del síndrome de Bland, White y Garland es difícil mediante el método clínico debido a la diversidad en su presentación. La prevalencia estimada en Cuba es mayor que lo registrado en el mundo actual. El diagnóstico temprano y tratamiento oportuno mejora de forma significativa la supervivencia y el pronóstico de los pacientes aquejados por esta dolencia(AU)

Introduction: The anomalous origin of left coronary artery that originates in the pulmonary artery, known as Bland, White and Garland is a congenital heart disease, with low incidence, variable clinical spectrum and is not exclusive of the pediatric age group. It has a high mortality in the first year of life if not diagnosed and treated early. Objective: To describe the characteristics of the diagnosis of Bland, White and Garland syndrome. Methods: It was made the characterization of the entity in Cuba in a decade (2005 to 2015). There were included all the patients with clinical manifestations suggestive to Bland, White and Garland syndrome and echocardiographic confirmation. It was made the calculation of this entity´s prevalence and it was used the percentage in the qualitative variables and Fisher's exact test. The statistical validation of research results had a significance level lower than 5 percent. Results: The estimated prevalence in that decade in Cuba was around 2.9 per 300 000 live births. It was demonstrated the variability in its clinical manifestations and wide range of onset´s age. The treatment is surgical with excellent survival and prognosis. Conclusions: The diagnosis of Bland, White and Garland syndrome is difficult through the clinical method due to the diversity in its presentation. The estimated prevalence in Cuba is greater than what was registered in the world today. Early diagnosis and prompt treatment significantly improves the survival and prognosis of patients suffering from this condition(AU)

Anomalous origin of the left coronary artery from the pulmonary artery: A surgical certainty.

This study aims to assess the clinical presentation and the outcomes of a surgical correction of an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). A retrospective review was carried out on the charts of six patients admitted for ALCAPA surgery at Aga Khan University Hospital, Karachi from March 2017 to May 2018.Dyspnoea, palpitation, poor feeding, fatiguability, pallor and a murmur of mitral regurgitation were the main presenting features. The pre-operative median left ventricular ejection fraction (LVEF) was 64%. Coronary reimplantation was performed in all the patients with a mitral valve repair being done in only one patient. The mea n LV EF was 66 .3%,p ost- ope rativel y. Mitra l regurgitation (MR) improved in patients post-operatively with trace in 2 patients and mild MR in one. Surgical correction by coronary re-implantation yields favourable outcomes in ALCAPA and significantly reduces the morbidity and mortality rates associated with the disease.

123I-BMIPP/201Tl dual myocardial SPECT proves the efficacy of surgical treatment for an adult with Bland-White-Garland syndrome.

Bland-White-Garland (BWG) syndrome is a rare congenital heart disease in which the left coronary artery originates from the pulmonary artery (PA). Surgical treatment to rebuild a dual coronary system is recommended at the time of the diagnosis. However, no effective operative procedure has been established for adult-type BWG patients because of the paucity of such cases. We herein report a case of adult-type BWG that was successfully treated by patch closure of the orifice of the left main tract from the main PA and coronary artery bypass grafting. 123I-15-(p-iodophenyl)-3-(R,S)-methylpentadecanoic acid (BMIPP) and 201thallium (Tl) dual myocardial single-photon emission computed tomography (SPECT) were performed before surgery, early after surgery, and at three months after surgery. Before surgery, dual SPECT showed myocardial perfusion defects in the anterior and septal wall, which corresponded to the cardiovascular magnetic resonance imaging findings. Early after surgery, only 201Tl images demonstrated an improvement in the defect area. At three months after surgery, both the 201Tl and 123I-BMIPP imaging findings demonstrated an improvement in the defect area, which was correlated with the recovery of the left ventricular function. These results showed the effectiveness of this surgical approach for BWG syndrome. .

Late adult presentation of ALCAPA syndrome: need for a new clinical classification? A case report and literature overview.

BACKGROUND: Anomalous origin of the left main coronary artery from the pulmonary artery (ALCAPA) is a very uncommon congenital coronary artery anomaly, most commonly presenting in early infancy. Late adult presentation of ALCAPA syndrome is extremely rare. CASE SUMMARY: We present a case of a 76-year-old patient with first presentation of ALCAPA. The coronary anomaly was first diagnosed during elective coronary angiography. The case was discussed at the Heart Team meeting and as the patient was asymptomatic, had good coronary collateral circulation, a medical treatment strategy was chosen and the patient was discharged in a good physical condition. During 3 years of follow-up, the patient has experienced no cardiovascular complications. DISCUSSION: We hereby also discuss briefly the clinical presentation, epidemiology, diagnostics and treatment options for adults with newly diagnosed ALCAPA and discuss the need for a new clinical classification. Only a few cases have been published of septuagenarians or octogenarians with first presentation of ALCAPA. To our knowledge, the patient presented in our case was one of the least symptomatic patients during her eight decades of life.

Long-term assessment of left ventricular ejection fraction and mitral regurgitation following takeuchi repair

Abstract Objective: This study aimed to evaluate the early operative outcomes and to compare the left ventricle and mitral valve functions after initial Takeuchi repair in patients with anomalous left coronary arising from pulmonary artery (ALCAPA). Methods: Fourteen patients (5 males, 9 females; mean age 4.3 years, ranging from 25 days to 34 years) who were operated for ALCAPA between 2007 and 2018 were included in this study. Data were evaluated retrospectively based on our medical records. Results: Hospital mortality rate was 7.1% (n=1). Thirteen surviving patients were kept in follow-up mean 4.3±3.05 years. When compared to preoperative measurements, both left ventricular ejection fraction (LVEF), (P=0.007) and mitral regurgitation (MR) (P=0.001) significantly improved before discharge. Moreover, LVEF values were improved in the late follow-up, considering early postoperative outcomes, and this alteration was significant (P=0.014). Nevertheless, alteration in the degree of MR among patients did not differ in the long-term follow-up (P=0.180). There was no late-term mortality or need for reoperation among patients. Conclusion: Although some centers prefer to direct implantation in ALCAPA, Takeuchi procedure can be accepted as a reliable method that provides satisfactory long-term results, considering that it aids to improve left ventricle ejection fraction and reduced mitral valve regurgitation.

Long-Term Assessment of Left Ventricular Ejection Fraction and Mitral Regurgitation Following Takeuchi Repair.

OBJECTIVE: This study aimed to evaluate the early operative outcomes and to compare the left ventricle and mitral valve functions after initial Takeuchi repair in patients with anomalous left coronary arising from pulmonary artery (ALCAPA). METHODS: Fourteen patients (5 males, 9 females; mean age 4.3 years, ranging from 25 days to 34 years) who were operated for ALCAPA between 2007 and 2018 were included in this study. Data were evaluated retrospectively based on our medical records. RESULTS: Hospital mortality rate was 7.1% (n=1). Thirteen surviving patients were kept in follow-up mean 4.3±3.05 years. When compared to preoperative measurements, both left ventricular ejection fraction (LVEF), (P=0.007) and mitral regurgitation (MR) (P=0.001) significantly improved before discharge. Moreover, LVEF values were improved in the late follow-up, considering early postoperative outcomes, and this alteration was significant (P=0.014). Nevertheless, alteration in the degree of MR among patients did not differ in the long-term follow-up (P=0.180). There was no late-term mortality or need for reoperation among patients. CONCLUSION: Although some centers prefer to direct implantation in ALCAPA, Takeuchi procedure can be accepted as a reliable method that provides satisfactory long-term results, considering that it aids to improve left ventricle ejection fraction and reduced mitral valve regurgitation.

Anomalous origin of the left coronary artery from the pulmonary artery in a patient of advanced age: a case report and analysis.

A 61-year-old Chinese man presented with a nearly 30-year history of an anomalous origin of the left coronary artery. He had been diagnosed with an anomalous origin of the left coronary artery in 1989. He then underwent regular echocardiographic examinations and it was found that his heart was gradually enlarging. After a >20-year asymptomatic period, he developed recurrent chest discomfort and palpitation. Coronary computed tomography angiography suggested that the left coronary artery anomaly originated from the pulmonary artery; additionally, the right coronary artery was tortuous and thickened. Coronary angiography showed that the right coronary artery was huge and buckling. The patient underwent corrective surgery of the anomalous origin of the left coronary artery from the pulmonary artery, aortic valve mechanical valve replacement, mitral valve plasty, and tricuspid valve plasty in Fuwai Hospital (National Center of Cardiovascular Disease of China), and the anatomic results of the surgery were good.

Unusual cause of left ventricular dysfunction in a child. / Causa rara de disfunção ventricular esquerda em idade pediátrica.

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery is a rare congenital heart disease and a cause of myocardial ischemia during childhood. Most undiagnosed cases die in the first year of life as an extensive collateral network is essential for survival. The diagnosis requires a high index of clinical suspicion. The authors present the case of an 8-year-old black asymptomatic child referred from Cape Verde Island in order to clarify left ventricular dilatation and dysfunction with systo-diastolic turbulent flows observed at the interventricular septum. At the age of 3 months, she was diagnosed with heart failure, in the context of showing dilated cardiomyopathy. She was managed and clinically improved with anticongestive therapy, which she was still taking at the time of admission to our Center. The echocardiogram findings suggested Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery and the diagnosis was confirmed by computerized angiotomography and cardiac catheterization. The patient was successfully submitted to direct implantation of the left coronary artery into the aorta, allowing the creation of a double coronary perfusion system. This case illustrates an unusual presentation of a rare pathology that survived without a diagnosis after the first year of life. It also reinforces the importance of multimodality image screening in these cases.

Predictors of Short-term Outcomes Following Repair of Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery in Chinese Children: A Case-Control Study.

OBJECTIVE: To analyze the predictive factors associated with prolonged mechanical ventilation (PMV) and prolonged duration of inotropic support (PDIS) following anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) surgery with possible concomitant mitral valve procedure (MVP) in a relatively young population. DESIGN: A retrospective case-control study. SETTING: Fuwai hospital. PARTICIPANTS: Pediatric patients with ALCAPA surgery from July 2010 to October 2017. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: PMV was defined as a mechanical ventilation time ≥48 hours and PDIS as a duration of inotropic support ≥6 days. Univariate analysis, logistic regression analysis, and receiver operating characteristic curve analysis were used to identify independent predictors of PMV and PDIS. The independent predictors of PMV were found to be the left ventricular end-diastolic diameter z-score (LVEDDz), estimate of glomerular filtration rate (eGFR) and maximum vasoactive-inotropic score (VISm). The odds ratios and 95% confidence intervals for these predictors of PMV were the following: LVEDDz, 1.58 (1.19-2.09); eGFR, 0.96 (0.93-0.998); and VISm, 1.08 (1.01-1.17). The predictor of PDIS was LVEDDz at 1.65 (1.26-2.15). One patient died during hospitalization, and there was no reoperation. Thirty-seven patients received concomitant MVP with no hospital mortality. At discharge, mitral regurgitation (MR) had improved in all patients with MR. Patients with PMV were associated with more acute kidney injury, PDIS, and longer ICU and hospital stays. CONCLUSIONS: In relatively young individuals who received ALCAPA surgery and possible concomitant MVP, short-term outcomes are favorable. Based on their experience bias, the authors recommend combining LVEDDz, eGFR, and VISm to predict PMV and applying LVEDDz for PDIS.

Longa sobrevivência em paciente com síndrome de ALCAPA não corrigida: Relato de caso e revisão da literatura

A origem anômala da artéria coronária esquerda da artéria pulmonar (síndrome de Bland-White-Garland ou ALCAPA: anomalous left coronary artery from the pulmonary artery) é uma cardiopatia congênita rara. Apresenta predominantemente na infância e suas principais formas de apresentação são como isquemia miocárdica ou insuficiência cardíaca. A sobrevivência à idade adulta é bastante incomum. Se não tratada, a mortalidade por ALCAPA se aproxima ao 90% na infância, portanto o reconhecimento precoce e a correção cirúrgica são essenciais. Apresentamos um caso de uma mulher de 56 anos, que acompanha em nosso ambulatório com o diagnóstico de síndrome de ALCAPA, e com piora recente da classe funcional de dispnéia.

The anomalous origin of the coronary artery from the pulmonary artery (ALCAPA or White-Bland-Garland syndrome) is a rare congenital cardiopathy. It presents predominantly in childhood and its main presentation forms are myocardial ischemia and heart failure. The survival to adulthood is unusual. If not treated, mortality by ALCAPA syndrome is close to 90% in childhood, therefore, the early recognition and surgical repair are essential. We present the case of a 56 years old woman, followed by our ambulatory with the ALCAPA Syndrome diagnosis, and recent functional class and dyspnea worsening