11/329. Left superior vena cava connection to unroofed coronary sinus associated with positional cyanosis: successful transcatheter treatment using Gianturco-Grifka vascular occlusion device. A persistent left superior vena cava connection to an unroofed coronary sinus is a rare cardiac anomaly that is associated with a variable degree of cyanosis. We report an infant with this condition and the unusual feature of cyanosis dependent on head position. When the patient's head was rotated to the left, he developed severe stenosis of the left internal jugular vein, enlarged cervical collateral veins that connected to the right superior vena cava and had an oxygen saturation 95%. When the patient's head was rotated to the right, the left internal jugular vein was widely patent and systemic oxygen saturation decreased to 87%. There was no right ventricular volume overload. Temporary occlusion of the left superior vena cava documented tolerable proximal venous pressure. cyanosis was relieved by transcatheter closure of the left superior vena cava with a Gianturco-Grifka vascular occlusion device. Cathet. Cardiovasc. Intervent. 48:369-373, 1999. ( info) |
12/329. One day old infant with acyanotic congenital heart disease: critical aortic stenosis. Congenital aortic stenosis accounts for about 5% of cardiac malformations recognized in childhood. It belongs to the category of acyanotic congenital heart disease. These lesions produce a load on the heart because of left ventricular outflow tract obstruction. Severe aortic stenosis in the newborn period (critical aortic stenosis) presents with signs of left sided heart failure (pulmonary edema, poor perfusion), right sided heart failure (hepatomegaly, peripheral edema) and may progress rapidly to total circulatory collapse. We present a case of an infant with critical aortic stenosis presenting with cyanosis, who was entirely dependent on ductal patency for systemic output. When oxygen was given, the ductus started to close, with a worsening of the left sided output and subsequent acidosis. With the right to left shunt across the ductus, the baby was cyanotic and dependent on prostaglandin to keep the ductus open. There was minimal flow across the aortic valve because of the stenosis and extremely poor left ventricular function prior to surgery. After relief of the aortic valvular obstruction, there was finally good antegrade flow across the aortic valve, terminating cyanosis. ( info) |
13/329. Doppler echocardiographic evaluation of the hemodynamics in absent aortic valve. hemodynamics were estimated by Doppler echocardiography in a neonate with an absent aortic valve and absent or extremely hypoplastic mitral valve. The coronary blood flow depended on the increased end-diastolic pressure of the left ventricle. Pulmonary venous congestion, which was also due to the increased end-diastolic pressure of the left ventricle, may induce decreased oxygen saturation and, subsequently, further myocardial hypoxia, poor contraction and increased end-diastolic pressure of the left ventricle. Finally, hypoxic blood was supplied to each organ from the pulmonary artery through the ductus arteriosus, which induced severe acidosis and differential cyanosis after birth. ( info) |
14/329. Lupoid hepatitis, Rendu-Osler-Weber syndrome, clubbing cyanosis and hypertrophic osteoarthropathy. Chronic liver disease typical of chronic active 'lupoid' hepatitis together with cyanosis, clubbing and hypertrophic osteoarthropathy in a 42-year-old female is described. In addition she had severe nose bleeds, gastro-intestinal haemorrhages, syncopal attacks with generalised convulsive seizures, pulmonary arterio-venous fistulae as manifestations of Rendu-Osler-Weber syndrome. A study of the literature revealed that similar associations are far more frequent than can be attribtued to chance. Possible mechanisms of the cyanosis, clubbing and osteoarthropathy and possible common pathogenesis for these seemingly unrelated disorders are discussed. ( info) |
15/329. Device closure of an atrial septal defect following successful balloon valvuloplasty in a neonate with critical pulmonary valve stenosis and persistent cyanosis. Persistent cyanosis after successful balloon valvuloplasty for neonatal critical pulmonary valve stenosis is often related to poor right ventricular compliance and right-to-left shunting at the atrial level. A successful catheter closure of an atrial septal defect was performed with a dramatic increase in systemic oxygen saturation alleviating the need for a surgical systemic-to-pulmonary artery shunt. ( info) |
16/329. Primary transcatheter treatment of congenital pulmonary arteriovenous malformation causing cyanosis of the newborn. We report on successful transcatheter therapy of congenital pulmonary arteriovenous malformation resolving persistent cyanosis and obviating the need for surgical pneumonectomy or lobectomy. ( info) |
17/329. Transient neonatal cyanosis: unusual presentation of right-sided cardiac masses. We report a case of a neonate with multiple cardiac masses, cyanosis, and a heart murmur. Arterial desaturation was the result of right-to-left shunting at the foramen ovale level caused by tricuspid regurgitation. Three right-sided cardiac masses were detected by echocardiography. By 2 weeks of age the patient had complete resolution of his cyanosis and improved tricuspid regurgitation following the normal decrease in pulmonary vascular resistance. At 2 years of age, he has no cardiovascular symptoms and the masses are calcified and have no hemodynamic consequences. ( info) |
18/329. Occlusion of azygos vein via direct percutaneous puncture of innominate vein following cavopulmonary anastomosis. A 2-year-10-month-old boy was diagnosed with a complex congenital heart disease: right atrial isomerism, left superior vena cava (LSVC), complete atrioventricular septal defect, secundum type atrial septal defect, transposition of the great arteries with pulmonary atresia, patent ductus arteriosus, absence of a right superior vena cava (RSVC), and dextrocardia. He had received a left Blalock-Taussig (BT) shunt at the age of 3 months and a left bidirectional Glenn shunt one year after BT shunt. Progressive cyanosis was noted after the second operation and cardiac catheterization showed a functional Glenn shunt with an engorged azygos vein, which was inadvertently skipped for ligation. Because of the absence of RSVC, transcatheter occlusion of the azygos vein was performed successfully via direct puncture of the innominate vein. ( info) |
19/329. cyanosis caused by a huge obstructive right ventricular fibroma. Cardiac fibromas are rare lesions which occur more often in infants and children than in adults. These tumors are benign proliferations of connective tissue most often found in the left ventricular myocardium or septum. In an 8-month-old infant with cyanosis and progressive exertional dyspnea, a huge cardiac tumor obstructing the right ventricular outflow tract (RVOT) was diagnosed by means of 2-dimensional echocardiography and cardiac catheterization. At surgery, a whitish gray solitary tumor measuring 5.0 x 4.5 cm could be well visualized. It was nearly totally resected, and the RVOT was reconstructed with an Equine pericardial patch. Histologic examination classified the tumor as a fibroma. Although surgical mortality in cardiac fibroma with RVOT obstruction is extremely high, early diagnosis and prompt excision of the tumor is mandatory in relieving its dangerous symptoms. ( info) |
20/329. Unusual respiratory response to oxygen in an infant with repetitive cyanotic episodes. High inspired oxygen concentrations have recently been recommended to control cheyne-stokes respiration in adults, with the intention of averting periodic apnea and its attendant arterial desaturation. We report a case study on an infant presenting with recurrent apnea and cyanosis in which oxygen treatment led to a gross form of respiratory instability we call episodic breathing, in which a breathing phase of 60 to 90 s alternated with an apnea lasting up to 60 s. When oxygen was discontinued, a profound arterial desaturation developed before breathing recommenced and restored oxygen levels. We propose that episodic breathing is an unusual respiratory pattern that involves the central chemoreceptors and results from the ventilatory threshold (the central PCO(2) at which breathing starts) lying considerably above the apneic threshold (the central PCO(2 )at which breathing stops). This feature predisposes to lengthy periods of hyperpnea alternating with lengthy periods of apnea. We suggest that when the case infant returned to air during episodic breathing, termination of apnea was entirely dependent upon carotid body activity, which reached a sufficient level to restart breathing only when arterial desaturation was severe. We conclude that oxygen therapy involves potential risks when employed to treat respiratory disorders involving unstable breathing patterns in the infant. ( info) |