Tetralogy of fallot with pulmonary atresia and a major aortopulmonary collateral artery (MAPCA) in a young adult: A case report
Philippine Journal of Internal Medicine
;
: 38-2010.
Artigo
em Inglês
| WPRIM
| ID: wpr-633339
ABSTRACT
CLINICAL PRESENTATION This report describes a case of a 24-year-old, acyanotic, female patient presenting with mild effort dyspnea, a right ventricular heave, a displaced cardiac apex, a loud single S2, a holosystolic murmur on the left parasternal area, and a continuous murmur at the left posterior chest. Transthoracic and transesophageal echocardiography demonstrated dilated left atrium and ventricle, hypertrophied right ventricle and a large ventricular septal defect with overriding of the aorta. However, both studies failed to visualize the pulmonic valve. The patient underwent cardiac catheterization which revealed the presence of pulmonary atresia. A descending aortogram showed the presence of a single large major aortopulmonary collateral artery (MAPCA) arising from the descending thoracic aor ta which supplies confluent left and right pulmonary arteries. The pulmonary arterial vasculature is enlarged and pruned in appearance. The main pulmonary artery is non-atretic. Mean proximal pulmonary artery pressure was 68 mmHg, indicating severe pulmonary hypertension. Patient wasstarted on digoxin and sildenafil.SIGNIFICANCE:
This case report emphasizes the importance of a well-developed central pulmonary circulation and an adequate but not excessive pulmonary blood supply from an aortopulmonary vascular connection in the survival of patients with pulmonary atresia into adulthood.
Texto completo:
DisponíveL
Índice:
WPRIM (Pacífico Ocidental)
Assunto principal:
Aorta
/
Valva Pulmonar
/
Tetralogia de Fallot
/
Cateterismo Cardíaco
/
Circulação Pulmonar
/
Atresia Pulmonar
/
Dispneia
/
Átrios do Coração
/
Comunicação Interventricular
/
Hipertensão Pulmonar
Limite:
Adulto
/
Feminino
/
Humanos
Idioma:
Inglês
Revista:
Philippine Journal of Internal Medicine
Ano de publicação:
2010
Tipo de documento:
Artigo
Similares
MEDLINE
...
LILACS
LIS