Antenatal Detection of Treatable Critical Congenital Heart Disease Is Associated with Lower Morbidity and Mortality.

OBJECTIVE: To establish the impact that timing of diagnosis and place of birth have on neonatal outcomes in those with readily treatable critical congenital heart disease. STUDY DESIGN: This was a population-based study with a complete national cohort of live-born infants with transposition of the great arteries and aortic arch obstruction in New Zealand between 2006 and 2014. Timing of diagnosis, place of birth, survival to surgery, in-hospital events, and neonatal mortality were reviewed. Live births with a gestation of ≥35 weeks and without associated major extracardiac anomalies were included for analysis. RESULTS: A total of 166 live-born infants with transposition of the great arteries and 87 with aortic arch obstruction were included. Antenatal detection increased from 32% in the first 3 years to 47% in the last 3 years (P = .05). During the same period, neonatal mortality decreased from 9% to 1% (P = .02). No deaths occurred after surgical intervention. An antenatal diagnosis was associated with decreased mortality (1/97 [1%] vs 11/156 [7%]; P = .03) and birth outside the surgical center was associated with increased risk of mortality (11/147 [7%] vs 1/106 [1%]; P = .02). Those with an antenatal diagnosis required fewer hours of mechanical ventilation (P = .02) and had shorter durations of hospital stay (P = .05) compared with those diagnosed >48 hours after birth. CONCLUSIONS: The mortality risk for transposition of the great arteries and critical aortic arch obstruction is greatest before cardiac surgery. Improved antenatal detection allowing delivery at a surgical center is associated with reduced mortality.

[Double aortic arch associated with stenosis of left pulmonary artery]. / Duplo arco aórtico associado a estenosa da artéria pulmonar esquerda.

Case report on a 6-year-old patient with vascular ring, due to double aortic arch. The patient was asymptomatic. The barium-filled esophagus showed bilateral indentations and a large posterior indentation. Echocardiographic study in notch suprasternal view revealed features of this pathology. The diagnosis was made by the cineangiocardiographic study that showed double aortic arch with separate carotid and subclavian arteries arising from each arch. The right arch was larger than the left, and the descending aorta was on the left side. There was severe left pulmonary artery stenosis associated. The clinical management was chosen, because the patient was asymptomatic.

Duplo arco aórtico associado a estenose da artéria pulmonar esquerda / Double Aortic Arch Associated With Left Pulmonary Artery Stenosis

Menino de 6 anos de idade assintomático, foi submetido a estudo radiológico com esôfago contrastado, A compressäo bilateral e posterior do esôfago era sugestiva de anel vascular formado por duplo arco aórtico. O estudo ecocardiográfico, em corte supra-esternal evidenciou achados sugestivos desta malformaçäo. O diagnóstico foi feito pelo estudo cineangiocardiográfico que mostrou duplo arco aótico, com as artérias carótida e subclávia direitas nascendo do arco direito e as artérias carótida e subcláva esquerdas nascendo do arco esquerdo. O arco direito era predominante e a aorta descendente localizava-se à esquerda. Existia também estenose da artéria pulmonar esquerda. Por ser assitnomático, optou-se pelo acompanhamento clínico

[Cardiac damage in Takayasu's arteritis. Study in 125 patients]. / Daño cardíaco en la arteritis de Takayasu. Estudio en 125 pacientes.

In order to asses the importance of cardiac damage in Takayasu's arteritis, 125 cases were studied and followed for 5.8 +/- 5.5 years. The arterial lesion involved the aorta and the principal abdominal branches in 10.4% of cases. Isolated lesions of the supraaortic vessels were present in 25.6% of cases. The rest of cases had obstructions in both arterial territories (64%). Cardiac damage was present in 82.4% of cases with the following manifestations: precordial murmurs (65%), cardiac enlargement (70%), heart failure (28%), angor pectoris (13.6%), abnormal electrocardiogram (60%): left ventricular hypertrophy (40.8%), right ventricular hypertrophy (8.8%) and conduction defects (12%). Aortic regurgitation secondary to enlargement of the aortic root was seen in 11.2%. Mitral incompetence due to left ventricular enlargement was documented in 13.6% of cases. In 2 patients rheumatic heart disease was associated to Takayasu's arteritis. Mortality was 4.8%; mostly due to congestive heart failure. The high incidence of cardiac damage was attributed to systemic arterial hypertension secondary to renovascular obstructions or coarctation of the aorta. Aortic and mitral regurgitation, pulmonary hypertension, and coronary arterial lesions contributed to cardiac damage.