Cases reported "Heart Defects, Congenital"

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1/100. Progressive pulmonary autograft root dilatation and failure after Ross procedure.

    We present a case of progressive pulmonary autograft root dilatation and subsequent failure after a Ross procedure. The explanted autograft vessel wall revealed striking histologic findings indicative of chronic media rupture. Examination of another explanted pulmonary autograft root showed similar histologic changes, suggesting a common phenomenon in pulmonary autograft roots. It may be the cause of progressive root dilatation as observed after Ross operations.
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2/100. A detailed histologic analysis of pulmonary arteriovenous malformations in children with cyanotic congenital heart disease.

    INTRODUCTION: Pulmonary arteriovenous malformations are a common cause of progressive cyanosis in children after cavopulmonary anastomoses. We analyzed the pulmonary histologic characteristics from children in whom pulmonary arteriovenous malformations developed after procedures that resulted in pulmonary arterial blood flow devoid of hepatic venous effluent. methods: We performed routine histologic studies, immunohistochemical staining, and electron microscopic analysis of peripheral lung biopsy specimens from 2 children with angiographically proven pulmonary arteriovenous malformations. Microvessel density was determined with a computer-assisted, morphometric analysis system. RESULTS: Histologic examination demonstrated large, dilated blood vessels ("lakes") and clustered, smaller vessels ("chains") in the pulmonary parenchyma. Microvessel density was significantly greater in these patients than in age-matched controls (P =.01). immunohistochemistry demonstrated uniform staining for type IV collagen and alpha-smooth muscle actin, weak staining for the endothelial marker CD31 (cluster of differentiation, PECAM-1), and negative staining for proliferating cell nuclear antigen. Electron microscopy revealed endothelial irregularity, a disorganized basement membrane, and increased numbers of collagen and actin filaments beneath the endothelium. CONCLUSIONS: This study represents an attempt to characterize the histologic features of pulmonary arteriovenous malformations in children with congenital heart disease who have pulmonary arterial blood flow devoid of hepatic venous effluent. The histologic correlate of this condition appears to be greatly increased numbers of thin-walled vessels. immunohistochemistry suggests that the rate of cellular proliferation is not increased in these lesions. The development of these techniques may provide a standardized histologic approach for this condition and aid in understanding its etiology.
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3/100. Acardiac acephalus: a case report and implications on expectant management.

    Acardiac acephalus is a rare occurrence resulting from extensive anastomoses between the vessels of the monochorionic twins which invariably leads to death of the "perfused" twin and an estimated 50% perinatal mortality rate in the "pump" twin, due mainly to premature delivery or congestive heart failure. Once diagnosis has been documented, blocking the vascular flows between the twins has been the principle goal of treatment with the assumption that a more favorable chance of survival in the pump twin can be achieved. However, hasty surgical intervention without a well-designed preoperative evaluation may result in unfavorable outcomes. A 21-year-old nullipara was found to carry an acardiac parabiotic twin in a monochorionic twin pregnancy at 28 weeks of gestation and was referred to us from a local clinic. Expectant management based on the close monitoring of the well-being of the healthy co-twin is presented in this article along with review of published works on the management of this rare condition.
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4/100. Fetal diagnosis of common arterial trunk with interrupted aortic arch using color power Doppler angiography.

    We successfully visualized the brachiocephalic arteries and aortic arch in a fetus seen at 19 weeks of gestation with a common arterial trunk and interrupted aortic arch by means of color power Doppler angiography, a new diagnostic development of color Doppler echocardiography. Power Doppler imaging is more sensitive to the state of low flow in fetal vessels, thus providing better visualization of fetal vascular structures from an early gestational stage.
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5/100. In utero development of hypertensive necrotizing pulmonary arterial lesions: report of a case associated with premature closure of the ductus arteriosus and pulmonary hypoplasia.

    Premature closure of the ductus arteriosus (PCDA) is an uncommon defect in which pulmonary hypertension (PH) has been documented by echocardiography in patients and by direct measurement after experimental PCDA in animals. The pulmonary vascular histology in human cases has received little attention but in the few recorded observations the vessels were either normal or showed increased muscularity. We report the case of a 31 week hydropic female stillborn monozygotic twin in whom postmortem examination disclosed PCDA and hypoplasia of the lungs. Atypical plexiform lesions with necrotizing pulmonary arteritis were present. These lesions represent vascular consequences of severe pulmonary hypertension produced by greatly enhanced blood flow through a restricted vascular bed resulting from the combined effects of these two abnormalities. The findings in this case of PCDA with presumed severe PH indicate that severe pulmonary vascular changes can develop in utero and that the interval of time needed for development of such chances in secondary PH is relatively short.
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6/100. Persistent left superior vena cava: case report and literature review.

    A persistent left superior vena cava (PLSVC) is the most common thoracic venous anomaly. It is a persistent remnant of a vessel that is present as a counterpart of normal right-sided superior vena cava (SVC) in early embryological development but normally disappears later. Although it can be easily diagnosed by the characteristic chest radiographic appearance of a pulmonary artery catheter (PAC) passed through it after being inserted into the left subclavian or jugular vein, its diagnosis is usually missed by the presence of normal (right) SVC and the passage of the catheter on the right side. Its diagnosis can be confirmed by many noninvasive and invasive tests, or it is incidentally diagnosed at thoracic surgery or autopsy. If it is not associated with other congenital cardiac anomalies, it is usually asymptomatic and hemodynamically insignificant. However, PLSVC has important clinical implications in certain situations. In this article, we describe a patient with bilateral SVC (a normal right SVC and a PLSVC) identified by a PAC in the PLSVC and the pacemaker wires in the right SVC. In addition, we review the literature on prevalence, embryological development, diagnosis, and clinical implications of PLSVC.
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7/100. diagnosis of left juxtaposition of the atrial appendages in the fetus.

    We describe two cases of left juxtaposition of the atrial appendages in which fetal echocardiograms provided a clue to the diagnosis. Both cases were associated with complex cyanotic congenital heart disease. The clue to the diagnosis was found at the three-vessel view. Abnormal vascular spaces were seen on the left side of the cross-sections of the great arterial trunks.
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8/100. Ivemark syndrome: asplenia with kidney collecting duct cysts and polysplenia with cerebellar cyst.

    Two newborns, one male and one female, from two different families, with Ivemark syndrome proven at autopsy are reported. One of them had asplenia and another had polysplenia. Both newborns had complex cardiac defects with isomerism of the lungs. The newborn with asplenia had dextrocardia, transposition of the great vessels, stenosis of the pulmonary artery, common atrioventricular canal and patent ductus arteriosus. The newborn with polysplenia had a common atrium, hypoplastic left ventricle and patent ductus arteriosus. The patient with asplenia had cystic dilated collecting ducts of the kidney and the patient with polysplenia had cerebellar cyst. These associate malformations have not been reported previously. Both cases were sporadic.
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9/100. Bifurcation of the left superior pulmonary vein, associated with an anomalous connection with the left atrium.

    An unusual case of bifurcation of the left superior pulmonary vein (LSPV) just before it enters the pericardium is described. The LSPV, which at the hilus of the lung originated from normal confluence of a superior and inferior root, bifurcated near the left atrium (LAt) of the heart into anterior (AB) and posterior (PB) branches that, separately invaginating the parietal pericardium, formed two individual serous sheaths. The PB coursed almost horizontally and opened, as usual, into the supero-dorsal wall of the LAt. The AB turned downward, reached the superior margin of the left auricle (LAu) and emptied into it. Thus, the AB was interposed between the pulmonary trunk and the LAt obstructing on the left side the communication between the transverse sinus of the pericardium and the pericardial cavity. The auricular opening of the AB was avalvular, but, unlike those of the normal pulmonary veins (PVs) which are surrounded by a large smooth inner surface, was, except for a narrow smooth-walled zone, close to the pectinate muscles. Moreover, an inferior muscular ridge at the inferior margin of its orifice of entrance into the LAu, separated it from the cavity of the LAt. It is well known that in development the PVs arise from convergence of capillaries belonging to the mediastinal part of the primitive splanchnic plexus and drain this into the systemic (cardinal and vitello-umbilical) veins of the embryo. As a consequence, it might be hypothesized that the AB of the LSPV probably represents a partial remnant either of a pulmonary-cardinal anastomotic mediastinal vein, or of a diverging vessel of the mediastinal plexus from which the PVs originate. In either case the AB became absorbed by the LAu, which, while it was developing on the left side of the primitive truncus arteriosus, drew the AB forward and downward, in the direction of its movement. The influence of such an anomaly of the PVs for altered intracardiac hemodynamics of the oxygenated blood flow has to be emphasized. Furthermore, the particular location of the AB, obstructing the communication between transverse sinus and pericardial cavity, can be a hindrance during cardio-pulmonary surgery.
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10/100. Severe polyhydramnios in twin reversed arterial perfusion sequence: successful management with intrafetal alcohol ablation of acardiac twin and amniodrainage.

    We present two cases of twin reversed arterial perfusion (TRAP) sequence complicated by severe polyhydramnios during the second trimester. Both cases were successfully managed at 27 and 26 weeks with ultrasound-guided intrafetal alcohol ablation of the acardiac twin and amniodrainage. The pump twins were delivered at 35 and 37 weeks, respectively, and both had uneventful postnatal courses. These cases suggest that, in the setting of severe polyhydramnios, prenatal intervention in TRAP sequence could certainly improve the otherwise poor prognosis of the pump twin. Targeting the main intra-abdominal vessel of the acardiac twin rather than its umbilical cord seems to be a good alternative in the prenatal treatment of these cases. The option of percutaneous intrafetal alcohol injection is widely available and less invasive, simpler and easier to perform than recently advocated endoscopic techniques.
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