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)

Epidemiology of acute kidney injury among paediatric patients after repair of anomalous origin of the left coronary artery from the pulmonary artery.

OBJECTIVES: Acute kidney injury (AKI) is a prevalent complication after the surgical repair of paediatric cardiac defects and is associated with poor outcomes. Insufficient renal perfusion secondary to severe myocardial dysfunction in neonates is most likely an independent risk factor in patients undergoing repair for anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). We retrospectively investigated the epidemiology and outcomes of children with ALCAPA who developed AKI after repair. METHODS: Eighty-nine children underwent left coronary reimplantation. The paediatric-modified risk, injury, failure, loss and end-stage (p-RIFLE) criteria were used to diagnose AKI. RESULTS: The incidence of AKI was 67.4% (60/89) in our study. Among the patient cohort with AKI, 23 (38.3%) were diagnosed with acute kidney injury/failure (I/F) (20 with acute kidney injury and 3 with acute kidney failure). Poor cardiac function (left ventricular ejection fraction < 35%) prior to surgery was a significant contributing factor associated with the onset of AKI [odds ratio (OR) 5.55, 95% confidential interval (CI) 1.39-22.13; P = 0.015], while a longer duration from diagnosis to surgical repair (OR 0.97, 95% CI 0.95-1.00; P = 0.049) and a higher preoperative albumin level (OR 0.83, 95% CI 0.70-0.99; P = 0.041) were found to lower the risk of AKI. Neither the severity of preoperative mitral regurgitation nor mitral annuloplasty was associated with the onset of AKI. After reimplantation, there was 1 death in the no-AKI group and 2 deaths in the AKI/F group (P = 0.356); the remaining patients survived until hospital discharge. The median follow-up time was 46.5 months (34.0-63.25). During follow-up, patients in the AKI cohort were seen more often by specialists and reassessed more often by echocardiography. CONCLUSIONS: Paediatric AKI after ALCAPA repair occurs at a relatively higher incidence than that suggested by previous reports and is linked to poor clinical outcomes. Preoperative cardiac dysfunction (left ventricular ejection fraction < 35%) is strongly associated with AKI. The beneficial effect of delaying surgery seen in some of our cases warrants further investigation, as it is not concordant with standard teaching regarding the timing of surgery for ALCAPA.

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.

Prevalence and characteristics of coronary artery anomalies detected by coronary computed tomography angiography in 5 634 consecutive patients in a single centre in Switzerland.

STUDY PRINCIPLES: Coronary computed tomography angiography (CCTA) allows three-dimensional visualisation of the origin, course and ending of the coronary vessels with high spatial resolution, yielding an accurate depiction of coronary artery anomalies (CAAs). This study sought to determine the prevalence, incidence and characteristics of CAAs detected with CCTA in a single centre in Switzerland. METHODS: CAAs were retrospectively identified in 5 634 consecutive patients referred for CCTA between March 2007 and July 2015. Single coronary arteries, Bland-White-Garland syndrome, anomalous coronary arteries originating from the opposite site of the sinus of Valsalva (ACAOS) with an interarterial course and coronary artery fistulas were classified as potentially malignant CAAs. RESULTS: We identified 145 patients with CAAs, resulting in an overall prevalence of 2.6% and cumulative incidence of 2.1% in all patients referred for CCTA in the observation period. Forty-nine (33.8%) patients showed malignant CAAs including 1 (0.7%) patient with Bland-White-Garland syndrome, 7 (4.8%) with single coronary arteries, 36 (24.8%) with ACAOS and an interarterial course, and 5 (3.5%) with coronary artery fistulas. The remaining 96 (66.2%) patients were classified as having benign variants. CONCLUSIONS: The prevalence of CAA detected by CCTA is not negligible. Because of its noninvasive nature, relatively low cost and low radiation exposure, a further increase in the utilisation of CCTA may be expected, which may consequently be paralleled by an increasing absolute number of incidentally detected CAAs. Hence, awareness of the main issues and possible management strategies regarding CAAs is of importance for every treating physician.