Hipertensión arterial pulmonar y cirugía de switcharterial neonatal para la corrección de la transposición de grandes arterias / Pulmonary arterial hypertension and neonatal arterial switch surgery for correction of transposition of the great arteries

Introducción y objetivos: Son escasas las publicaciones sobre aparición de hipertensión arterial pulmonar tras la cirugía de switch arterial en periodo neonatal para la corrección de la transposición de grandes arterias. Se evalúa la frecuencia y el comportamiento clínico de esta complicación en una serie de pacientes. Métodos: Se revisó la base de datos y se seleccionó a pacientes con transposición de grandes vasos corregida con switch arterial neonatal en el centro en los que con el tiempo apareció hipertensión pulmonar. Resultados: Se halló a 2 pacientes (1,3%) con transposición de grandes arterias corregida con éxito en la primera semana de vida que luego presentaron hipertensión arterial pulmonar. El primero es una niña de 7 años con diagnóstico de hipertensión pulmonar grave a los 8 meses de edad, sin respuesta a tratamiento médico, que precisó trasplante pulmonar. La anatomía patológica mostró hallazgos compatibles con hipertensión arterial pulmonar grave. El segundo es un niño de 24 meses con diagnóstico de hipertensión pulmonar grave a los 13 meses, sin respuesta al tratamiento médico. Conclusiones: La hipertensión arterial pulmonar es una complicación infrecuente pero muy grave cuya aparición se debe investigar en todo paciente con transposición de grandes vasos sometido a operación de switch arterial neonatal con el fin de instaurar un tratamiento agresivo temprano para los pacientes afectados, dados la escasa respuesta al tratamiento y el mal pronóstico que supone (AU)

Introduction and objectives: There are few reports of the appearance of pulmonary arterial hypertension following arterial switch surgery in the neonatal period to correct transposition of the great arteries. We assessed the frequency and clinical pattern of this complication in our series of patients. Methods: Our database was reviewed to select patients with transposition of the great arteries corrected by neonatal arterial switch at our hospital and who developed pulmonary hypertension over time. Results: We identified 2 (1.3%) patients with transposition of the great arteries successfully repaired in the first week of life who later experienced pulmonary arterial hypertension. The first patient was a 7-year-old girl diagnosed with severe pulmonary hypertension at age 8 months who did not respond to medical treatment and required lung transplantation. The anatomic pathology findings were consistent with severe pulmonary arterial hypertension. The second patient was a 24-month-old boy diagnosed with severe pulmonary hypertension at age 13 months who did not respond to medical therapy. Conclusions: Pulmonary hypertension is a rare but very severe complication that should be investigated in all patients with transposition of the great arteries who have undergone neonatal arterial switch, in order to start early aggressive therapy for affected patients, given the poor therapeutic response and poor prognosis involved (AU)

Pulmonary Arterial Hypertension and Neonatal Arterial Switch Surgery for Correction of Transposition of the Great Arteries.

INTRODUCTION AND OBJECTIVES: There are few reports of the appearance of pulmonary arterial hypertension following arterial switch surgery in the neonatal period to correct transposition of the great arteries. We assessed the frequency and clinical pattern of this complication in our series of patients. METHODS: Our database was reviewed to select patients with transposition of the great arteries corrected by neonatal arterial switch at our hospital and who developed pulmonary hypertension over time. RESULTS: We identified 2 (1.3%) patients with transposition of the great arteries successfully repaired in the first week of life who later experienced pulmonary arterial hypertension. The first patient was a 7-year-old girl diagnosed with severe pulmonary hypertension at age 8 months who did not respond to medical treatment and required lung transplantation. The anatomic pathology findings were consistent with severe pulmonary arterial hypertension. The second patient was a 24-month-old boy diagnosed with severe pulmonary hypertension at age 13 months who did not respond to medical therapy. CONCLUSIONS: Pulmonary hypertension is a rare but very severe complication that should be investigated in all patients with transposition of the great arteries who have undergone neonatal arterial switch, in order to start early aggressive therapy for affected patients, given the poor therapeutic response and poor prognosis involved.

Transposition of the Great Arteries in Fetal Life: Accuracy of Diagnosis and Short-Term Outcome.

OBJECTIVE: To review our series of prenatally diagnosed transposition of the great arteries (TGA) to analyze the accuracy of fetal echocardiography for achieving a precise diagnosis of the TGA type (simple vs. complex) and to examine the short-term outcome. METHODS: A total of 94 cases of simple and complex TGA types (ventriculoarterial discordance with atrioventricular concordance) prenatally evaluated in our referral center between 1998 and 2014 were included. Fetuses with additional congenital anomalies and those with incomplete follow-up were excluded. Prenatal diagnostic accuracy and short-term survival were analyzed for the different types of TGA. RESULTS: The TGA type was correctly ascertained prenatally in 93.3%. Most fetuses were diagnosed with simple TGA (62.7%). There were 6 discrepancies: 5 fetuses with simple TGA had postnatally TGA + ventricular septal defect (VSD; n = 3) or TGA + VSD + coarctation of the aorta (n = 2), and 1 fetus with TGA + VSD postnatally showed severe left ventricular outflow tract obstruction. The mortality rate was 6.6%; it was higher in complex versus simple forms (12.8 vs. 1.9%, p = 0.038), and in cases with intramural coronary artery versus those without (60 vs. 3.5%, p < 0.001). We found no relationship between the arrangement of the great arteries and coronary arterial abnormalities. CONCLUSIONS: Simple TGA has a better outcome than the complex forms. A discrepancy rate of 7% with potential influence on the prognosis of survival between the prenatal diagnosis of the TGA type and the definitive diagnosis was found.