Evaluación ecocardiográfica de origen anómalo de una rama arterial pulmonar desde la porción ascendente de la aorta (hemitruncus) / Echocardiographic evaluation of the anomalous origin of the pulmunary arterial branch from the ascending portion of the aorta (hemitruncus)

El origen anómalo de una de las ramas pulmonares desde la aorta ascendente en vez de ocurrir desde el tronco pulmonar, también conocido como hemitruncus constituye una singularidad cardiovascular congénita muy rara, pues ocurre en aproximadamente 0,05% de todas las forma de cardiopatías congénitas (¹). En la forma más frecuente depresentación de este defecto la rama pulmonar derecha (RPD) emerge directamente de la aorta ascendente en un 70%-80% de los casos (²). La ecocardiografía con Doppler color es el método de mayor utilidad para realizar el diagnóstico, los cortes apicales, paresternales y subcostales en eje corto de grandes vasos así como las visiones supraesternales longitudinales son esenciales para conseguir una completa evaluación del defecto. Presentamos una secuencia de imágenes ecocardiográficas de una paciente que es referida tardíamente a nuestro centro para evaluación cardiológica con historia de cianosis desde el nacimiento, fatiga y episodios repetidos de hemoptisis que resultó ser portadora de un origen anómalo de rama derecha de arterial pulmonar emergiendo desde la aorta ascendente y que desarrolló hipertensión arterial pulmonar en rangos suprasistémicos con resistencia vascular pulmonar también muy elevada.

Anomalous origin of one pulmonary branch from the ascending aorta rather than occurring from the pulmonary trunk, also known as hemitruncus is a very rare congenital cardiovascular entity, which occurs in approximately 0.05% of all forms of congenital heart disease (¹). In the most common presentation of this defect, the right pulmonary branch (RPB) emerges directly from the ascending aorta in 70%-80% of cases (²). Color Doppler echocardiography is the most useful method to establish the diagnosis. Apical, subcostal, and parasternal short axis views of the great vessels and longitudinal suprasternal views are essential for a complete assessment of the defect. We present a sequence of echocardiographic images of a patient referred late to our center for a cardiac evaluation with a history of cyanosis from birth, fatigue and repeated episodes of hemoptysis that resulted from having an anomalous origin of the right pulmonary artery branch emerging from the ascending aorta with development of pulmonary arterial hypertension exceeding systemic levels along with very high pulmonary vascular resistance.

Diagnosis of the Double Aortic Arch and Its Differentiation from the Conotruncal Malformations

PURPOSE: The clinical and radiological characteristics of the double aortic arch (DAA) and its differentiation from conotruncal malformations (CTM) were reported in order to familiarize pediatric practitioners with these congenital heart diseases. MATERIALS AND METHODS: From July 1994 to December 2006, a total of 6 patients (4 male and 2 female, aged 16 days to 6.5 years) with DAA were enrolled in this retrospective study. The study modalities included chart recordings, plain chest radiographs, barium esophagograms, echocardiograms, cardiac catheterization, cardiac angiograms, surgery, magnetic resonance imaging, and chromosome analysis. Patients with incomplete vascular rings or with right aortic arches and left ligamentum were excluded. In addition, the clinical and radiological profiles of 38 patients with CTM, including dextro-transposition of the great arteries (d-TGA) (n=28), hemitruncus arteriosus (HTA) (n=3), type I truncus arteriosus (TA) (n=4), and the aortopulmonary window (APW) (n=3), were comparatively reviewed. RESULTS: All 6 patients with DAA presented with postprandial choking and respiratory distress that prompted their initial visit to the hospital. One of the 6 patients presented with congestive heart failure, and none with cyanosis. Esophagograms showed indentations in 5 patients with DAA. All patients with d-TGA presented with cyanosis and heart failure, while patients with HTA, type I TA, and APW manifested overt heart failure. Suprasternal and subcostal approaches of the echocardiography may offer diagnositic windows for DAA. As for CTM, parasternal and subcostal approaches could always determine the causality. Cardiac catheterization with angiography comprehensively delineated the pathology. CONCLUSION: In case of postprandial choking and respiratory distress in neonates and infants, barium esophagograms can indicate the presence of DAA. Diagnosis of DAA and its differentiation from the CTM can be achieved by echocardiography, angiography, or magnetic resonance imaging.

Diagnóstico ecocardiográfico del origen anómalo de una rama de la arteria pulmonar de la aorta ascendente / Echocardiographic diagnosis of anomalous origin of one pulmonary artery from the ascending aorta

Objetivo: Presentar nuestra experiencia en el diagnóstico ecocardiográfico del origen anómalo de una rama de la arteria pulmonar naciendo de la aorta ascendente (OAAP) y analizar su presentación clínica, tratamiento y evolución con especial énfasis en los aspectos ecocardiográficos. Método: Revisamos retrospectivamente los expedientes clínicos, estudios de gabinete y hallazgos transoperatorios de los pacientes con diagnóstico ecocardiográfico de OAAP estudiados en el Hospital Infantil de México "Federico Gómez" de 1991 al 2002. Resultados: El estudio incluye 12 niños con OAAP. Siete fueron hombres. La edad media de presentación fue de 2 meses. Cuatro pacientes se diagnosticaron en la etapa neonatal, 3 en el primer año y 5 después del año. El diagnóstico se estableció prospectivamente por ecocardiografía en todos y se confirmó al cateterismo en 8 y a la cirugía en 9. El origen aórtico de la rama derecha se encontró en 10 casos y de la izquierda en 2. Las anomalías asociadas fueron PCA en 6, CIV en 2 y ventana aortopulmonar, discordancia atrioventricular, doble vía de salida ventricular derecha y tetralogía de Fallot en 1 caso respectivamente. Se operaron 9 casos. Un paciente falleció en el 6o día postoperatorio. Los restantes se encuentran en buenas condiciones sin estenosis en el sitio de la anastomosis. La cirugía no fue aceptada en un caso. Un paciente no fue candidato a cirugía por enfermedad vascular pulmonar (EVP) avanzada y otro está en espera de cirugía. Conclusiones: El diagnóstico del OAAP puede establecerse con exactitud mediante ecocardiografía. El cateterismo cardiaco rara vez es necesario para confirmar la morfología, pero es obligado en niños mayores con sospecha de EVP.

Objective: To present our experience in the echocardiographic diagnosis of anomalous origin of one pulmonary artery from the ascending aorta (AOPA). To analyze its clinical presentation, treatment and outcomes with special emphasis in the echocardiographic data. Method: We reviewed restrospectively the clinical, hemodynamic data, and surgical findings of patients with an echocardiographic diagnosis of AOPA studied in the Hospital Infantil of Mexico "Federico Gomez" from 1991 to 2002. Results: The study includes 12 children with AOPA; Seven were males. The average age at diagnosis was two months; 4 in neonatal period, 3 under 1 year and 5 older than 1 year. The diagnosis was established prospectively by echocardiography in all patients and it was confirmed by angiography in 8 and at surgery in 9. Ten had anomalous origin of rigth pulmonary artery. The associated anomalies were patent ductus arteriosus in 6, ventricular septal defect in 2 and aortopulmonary window, atrioventricular discordance, double outlet right ventricle and tetralogy of Fallot in one case each. Nine underwent corrective surgery of all the anomalies. One patient died on the sixth postoperative day; the remaining are in good condition without stenosis at the site of the anastomosis. Surgery was refused in one. One patient was not candidate to surgery due to advanced obstructive pulmonary vascular disease (OPVD) and one case is awaiting surgery. Conclusion: The diagnosis of AOPA may be established with precision through echocardiography. Cardiac catheterization is seldom needed to confirm morphology but is mandatory in older children with suspected of OPVD. (Arch Cardiol Mex 2003; 73:115-123).