RESUMEN
A bicuspid aortic valve (BAV) refers to a condition in which the valve has two cusps rather than three. Usually ignored, this manifests itself later in life. This bicuspid valve may manifest earlier in children with significant aortic stenosis because they have a severe left ventricular outflow tract restriction that worsens over time. This syndrome commonly results in congestive heart failure in newborns and early neonatal life. There can be a small amount of significant risk in pediatric patients having this BAV causing stenosis and being one of the reasons for sudden, unexpected death. Morphological differences result from a congenital cardiac abnormality called BAV. This paper emphasizes the importance of a multidisciplinary team in managing BAV and critical aortic stenosis and provides evaluation and treatment guidelines for both conditions. Transcatheter or surgical intervention is used for symptomatic individuals or those with a moderate to severe obstruction of the left ventricular outflow tract.
RESUMEN
The occurrence of MAPCAs (major aortopulmonary collateral arteries) with TOF (tetralogy of Fallot) and bilateral hypoplastic pulmonary arteries together is a rare condition. Patients are typically middle-aged men who usually present with acute signs of cardiac manifestations. The anomalies have survival up to the fourth decade of life and are fraught with clinical challenges. Additionally, various congenital syndromic associations, such as DiGeorge syndrome, are associated with these anomalies. We report an extremely rare case of a 41-year-old male who came with complaints of chest pain, dyspnea on exertion, and headaches. The patient had a previous history of tuberculosis and a rare combination of MAPCAs with TOF and bilateral hypoplastic pulmonary arteries, with a right-sided aortic arch with an aplastic left subclavian artery. The importance of the case comes from the need to perform surgery on a middle-aged male who was completely asymptomatic prior to this.
RESUMEN
A rare disorder called pulmonary hypoplasia is characterized by inadequate lung development, which frequently results in respiratory dysfunction and other related abnormalities. We present a case of an 11-month-old male child with left lung hypoplasia, absent left pulmonary artery, and ventricular septal defect (VSD). The child exhibited symptoms of cough and cold, with a history of recurrent respiratory tract infections since birth. Cardiovascular examination revealed a pan systolic murmur consistent with VSD, while respiratory examination indicated decreased air entry on the left side. Imaging studies confirmed the absence of the left pulmonary artery and left lung hypoplasia. Despite recommendations for VSD surgery, the child's parents declined surgical intervention, leading to discharge against medical advice. This case highlights the challenges in managing pulmonary hypoplasia, especially when accompanied by complex congenital heart defects, and underscores the importance of multidisciplinary care and parental involvement in decision-making.
RESUMEN
The abnormal origin of the left pulmonary artery (LPA) from the ascending aorta is a rare cardiac condition that is often associated with several other congenital defects. In this paper, we report the case of an infant who presented with recurrent infections and was prenatally diagnosed with tetralogy of Fallot (TOF). During echocardiography, various other cardiac defects such as ventricular septal defects (VSD), pulmonary stenosis (PS), and dilated right heart chambers were identified. Furthermore, cardiac catheterization revealed an anomalous origin of the LPA arising from the aorta associated with a narrow pulmonary annulus. Due to both conditions sharing a similar embryological course, the condition is commonly associated with a conotruncal defect known as DiGeorge syndrome. Together, the overall combination of cardiac anomalies is both unusual and unique. This case study explains the clinical associations, embryological origin, and surgical management of this condition in an infant.
RESUMEN
Congenital complete heart block (CCHB) is a rare and potentially life-threatening condition, often associated with maternal autoantibodies. We present the case of a one-year-old girl with recurrent respiratory symptoms, ultimately diagnosed with CCHB and congenital heart disease. She exhibited bradycardia and signs of congestive heart failure. A diagnostic workup revealed significant cardiac abnormalities, including dilated chambers, ventricular septal defect, and patent ductus arteriosus. Serological tests for maternal autoantibodies were negative. The child's parents opted for discharge without surgical intervention. This case underscores the importance of comprehensive evaluation and management strategies in patients with congenital heart block, particularly in resource-limited settings.