Cases reported "Ductus Arteriosus, Patent"

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1/16. Modified Starnes operation for neonatal Ebstein's anomaly.

    We report the case of a severely symptomatic neonate with Ebstein's anomaly. A modified Starnes operation was performed, but insufficient drainage of venous blood returning through thebesian veins caused overdistention of the right ventricle and severe left ventricular dysfunction. Urgent reestablishment of right ventricular-right atrial communication successfully resolved these problems.
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2/16. Echocardiographic features of adult tetralogy of fallot with natural palliative correction by patent ductus arteriosus.

    A thirty-year-old man with the diagnosis of the tetralogy of fallot and patent ductus arteriosus was admitted to our hospital because of a syncope. He reported no previous symptoms. We diagnosed adult tetralogy of fallot, which included all four characteristic anomalies: ventricular septal defect, overriding aorta, pulmonary artery stenosis, and right ventricular hypertrophy. The associated persistent ductus arteriosus and the presence of compensatory arteriovenous communications produced a continuous flow load on the left ventricle, which resulted in moderate left ventricular hypertrophy, but without symptoms of pulmonary congestion or cardiac decompensation. Anatomic diagnosis and hemodynamic assessment were established by transthoracic and transesophageal echocardiography, with incidental finding of a quadricuspid aortic valve. To the best of our knowledge, our case of the adult form of Fallot's tetralogy associated with both patent ductus arteriosus and quadricuspid aortic valve is the first one ever described. It is well known that patients with tetralogy of fallot who do not undergo operation in childhood have short survival, which depends predominantly on the degree of pulmonary artery stenosis and early development of collateral circulation to the lungs. Long-term persistence of natural aortopulmonary anastomosis with systemic collateral circulation to the lungs and remodeling of the heart, with better hemodynamic balance as well as the presence of mild pulmonary artery stenosis probably enhanced the survival of our patient.
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3/16. Congenitally complete heart block. Developmental aspects.

    Three cases of congenitally complete heart block are described of hearts in which other minor congenital malformations were not in themselves severe enough to disrupt the atrioventricular conduction system. The cases fitted well into the categorization of complete heart block suggested by Lev. Two exhibited lack of communication between the atrial and conducting tissues, the other had discontinuity of the penetrating atrioventricular bundle. In an attempt to explain why this discontinuity between different segments of the conducting tissues occurs, we re-examined several series of graded human embryos. This investigation suggested that the anulus fibrosus in the normal heart is derived from sulcus tissue of the atrioventricular junction, the endocardial atrioventricular cushions playing a minor role in the separation of atria from ventricles. The relationships between the sulcus tissues and the different components of the atrioventricular junctional area are discussed in terms of an explanation both for the existence of different types of congenitally complete heart block and for persistence of Mahaim (nodo-ventricular and nodo-fascicular) fibers.
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4/16. Congenital external carotid-jugular fistula: report of two cases and a review of the literature.

    Congenital arteriovenous communications involving the external carotid artery and the jugular vein(s) are exceptionally rare. We report two cases of congenital external carotid-jugular fistulae with clinical, radiological, and surgical features and a review of the literature. The prospects of endovascular treatment and the limits of surgery are discussed.
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5/16. Successful surgical ligation under intraoperative portal vein pressure monitoring of a large portosystemic shunt presenting as an intrapulmonary shunt: report of a case.

    We report a rare case of patent ductus venosus (PDV) with collapsed intrahepatic portal branches and an intrapulmonary shunt. Excellent improvement of the intrahepatic portal vein flow was achieved by ligating the large ductus venosus under intraoperative portal vein pressure (PVP) monitoring. A 3-year-old boy being followed up for hypergalactosemia at a local hospital was found to have mild lip cyanosis, exertional dyspnea, clubbed fingers, and mild liver dysfunction with high levels of transaminase and ammonia. cardiac catheterization indicated an intrapulmonary shunt with a ratio of 33%. Abdominal ultrasonography and computed tomography showed remarkable communication between the portal vein and the inferior vena cava. We performed laparotomy and successfully ligated the PDV under PVP monitoring. The PVP did not increase until the catheter was removed 7 days postoperatively. The patient's liver function test results returned to normal within 2 weeks. His serum galactose level was 0 mg/dl after drinking milk, and his SpO2 in room air and exertional dyspnea also improved. He was discharged 18 days after his operation, without any complications. We propose that ligation of a PDV under PVP monitoring could be a treatment of choice, bearing in mind that PDV is associated with collapsed intrahepatic portal branches.
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6/16. Total anomalous pulmonary venous connection with intact interatrial septum.

    The presence of an interatrial communication is considered an integral part of the diagnosis of total anomalous pulmonary venous connection and is believed to be essential for survival. We report a 9-month-old infant with obstructed supracardiac total anomalous pulmonary venous connection without an interatrial communication.
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7/16. Aortico-right ventricular tunnel and critical pulmonary stenosis: diagnosis by two-dimensional and Doppler echocardiography and angiography.

    An infant with aortico-right ventricular (AO-RV) tunnel and critical pulmonary stenosis presented with severe distress at birth. We present the clinical, echocardiographic, and angiographic features, correlated with autopsy findings. Also we discuss the differentiation from other AO-RV communications and a theory for the embryogenesis. AO-RV tunnel should be considered in the differential diagnosis of a critically sick newborn with cyanosis, a "to and fro" murmur, and signs of right heart failure. The correct diagnosis can be made echocardiographically by demonstrating the two ends of the tunnel connecting the aorta and a dilated RV, two normal coronary arteries, and obtaining high-velocity systolic and diastolic Doppler flow signals in the tunnel. Surgical repair of this lesion is possible, and early diagnosis and a modification of the surgical procedure may help survival.
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8/16. paraplegia after ductus ligation accompanied by injury to intercostal artery.

    paraplegia is a hitherto unreported complication of the surgical treatment of patent ductus arteriosus. This communication presents the case history of a 5-year-old girl in whom this serious complications developed after ductus ligation accompanied by injury to an intercostal artery.
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9/16. intestinal perforation associated with indomethacin treatment in premature infants.

    Within 9 months we observed intestinal perforations in three very low birth weight (VLBW) infants undergoing indomethacin treatment for symptomatic patent ductus arteriosus (sPDA). The three patients exhibited striking similarities in their clinical courses and predisposing factors. Although clinical and histological criteria did not differentiate the perforations from necrotising enterocolitis (NEC), a well-known entity in premature infants, these events were remarkable to us since we had observed no other cases of NEC in recent years. From animal experiments and pathophysiological data, a role for indomethacin in gastrointestinal ischaemic damage must be considered. This communication is not meant to discredit indomethacin treatment. However, awareness of potential complications and careful monitoring during treatment is warranted.
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10/16. pulmonary atresia with intact ventricular septum and ventriculocoronary communications: surgical significance.

    The first stage of a repair of pulmonary atresia with intact ventricular septum (type I) was attempted in a 2-day-old infant. At surgery, decompression of the hypertensive small right ventricle was followed by a sudden loss of myocardial contractility and death. Postmortem examination revealed a fistula with a large orifice in the right ventricular infundibulum that communicated directly with the left main coronary artery. Severe hypertensive changes indicative of abnormally high perfusion pressure were noted in the distal left coronary artery branches. The clinical course suggests that the effect of relieving right ventricular outflow obstruction was a reduction of left main coronary artery blood flow, resulting in fatal intraoperative myocardial ischemia. This unusual case draws attention to the anomalous ventriculocoronary communications often present in pulmonary atresia and their potential for limiting a successful surgical repair.
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