1/33. Sudden death due to ventricular septal defect.Ventricular septal defects (VSD) are usually considered non-life-threatening, usually closing spontaneously or causing symptoms of congestive heart failure, which can be surgically treated in time to save the patient's life. Despite the usually benign clinical course of VSD, serious arrhythmias occur in 16-31% of patients. Sudden death accounted for one-third of all deaths in a series of medically managed patients and occurred in 4.2% of patients in a study of VSD and arrhythmias. Cardiac hypertrophy is the common denominator in all cases reported in detail of VSD-associated sudden death. We have encountered four cases of sudden unexpected death from VSD in infants ranging in age from 1 week to 3 and (1/2) months. In each case there was cardiomegaly and in one case there was pulmonary arteriolar medial thickening, with extension of smooth muscle into small intralobular vessels. In half of our cases the attending physician was sued for malpractice. We believe that VSD in infants and young children are potentially life-threatening malformations which warrant careful clinical follow-up.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
2/33. Extrauterine pelvic arteriovenous malformation mimicking the clinical presentation of structural heart disease.A case is reported of an extrauterine pelvic arteriovenous malformation involving branches of the internal iliac arteries. cardiomegaly and a rough cardiac murmur were the clinical presentations mimicking a structural heart disease. A continuous bruit could only be detected by the diaphragm of the stethoscope applied firmly to the left lower abdomen. Multiple blood samplings from inferior vena cava, and iliac and femoral veins for determination of oxygen saturation may be necessary for suspected cases. However, selective arteriography remains the best method for diagnosing the presence, extent, and multiplicity of the lesions before surgery or percutaneous arterial embolization.- - - - - - - - - - ranking = 76.197193304191keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
3/33. Successful treatment of a neonate with vein of Galen aneurysmal malformation.We present a follow-up study on a 5-year-old boy who was born with vein of Galen aneurysmal malformation (VGAM) accompanied by extreme cardiovascular distress. He was successfully treated by the staged endovascular technique during the neonatal stage. Postoperative angiographic study confirmed complete occlusion of the VGAM and the establishment of normal brain perfusion. At 5 years of age, the child is growing normally without neurological deficits or developmental retardation. We suggest that the restoration of normal brain perfusion in neonates with VGAM can cause normal brain development.- - - - - - - - - - ranking = 5keywords = malformation (Clic here for more details about this article) |
4/33. Congenitally unguarded tricuspid valve orifice with a giant right atrium and a massive clot in an asymptomatic adult.Congenitally unguarded tricuspid valve orifice, a variant of tricuspid valve dysplasia, is a rare malformation with protean manifestations. This report describes an asymptomatic adult who, on echocardiographic examination ordered in view of an abnormal 12-lead surface electrocardiogram and plain chest X-ray, was found to have an unguarded tricuspid valve orifice with a giant right atrium (12 x 10 cm), intense spontaneous echo contrast and a large right atrial clot.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
5/33. Small aortic root as a cause of sudden death in a young adult: a controversial topic revisited.BACKGROUND: Sudden cardiac death over the age of 35 years is mostly due to coronary atherosclerosis, whereas under the age of 35 years, a variety of mainly congenital malformations prevail. However, hypoplasia of the aortic root in adults, first introduced by Laurie in 1968 as a cause of sudden cardiac death in adults, is never included. CASE REPORT: We present a case of a 29-year-old female who suddenly and unexpectedly collapsed during recreational bicycling. ventricular fibrillation was recorded, but resuscitation attempts failed, and the patient was declared dead about 1 h after the event. autopsy revealed cardiac hypertrophy with extensive scarring and a small aortic root (calculated inlet/outlet diameters=14.9/14.3 mm), without obstructive coronary artery disease or any other plausible cause for sudden cardiac death. CONCLUSIONS: The observations strongly suggest that small aortic root (less than 2 cm in diameter) provided the background for cardiac hypertrophy and, eventually, myocardial ischemia and ventricular fibrillation. Pathologists should be aware of this possibility while evaluating cases of sudden cardiac death without an obvious pathologic substrate.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
6/33. Bilateral cystic adenomatoid lung malformation type III--a rare differential diagnosis of pulmonary hypertension in neonates.Congenital cystic adenomatoid lung malformation (CCAM) is a rare disorder characterized by replacement of normal pulmonary tissue with cysts of variable size and distribution. Only sporadic cases of CCAM type III have been described that were stillborn or died in the early neonatal period. To our best knowledge we report the first infant with bilateral CCAM type III that was not ventilator-dependent for the first 10 weeks of life and survived until the age of 5 months. Postnatally the newborn presented with signs of only mild respiratory distress and chest radiograph did not show evidence of cystic lesions. In the following weeks the major clinical symptom was persistent pulmonary hypertension with severe tachypnea, O2 requirement and CO2 retention. Pulmonary hypertension did not respond to HFOV ventilation, NO ventilation or prostacyclin inhalations. CONCLUSIONS: (1) Persistent pulmonary hypertension can be a major clinical feature in neonates with bilateral CCAM type III. (2) A normal chest X-ray does not exclude the possibility of bilateral type III CCAM. (3) Computerized tomography appears to be superior to conventional chest X-ray imaging in detection of small cystic lesions in infants with CCAM type III. (4) In cases of prenatally suspected CCAM type III, parents should be informed that short-term survival is possible.- - - - - - - - - - ranking = 5keywords = malformation (Clic here for more details about this article) |
7/33. Rapidly progressive obstructive cardiomyopathy in infants with Noonan's syndrome. Report of two cases.Two patients with hypertrophic obstructive cardiomyopathy and Noonan's syndrome are presented. Both patients were found at postmortem examination to have gross malformation of the mitral valve and obliteration of the left ventricle due to muscle hypertrophy. Each case demonstrated similar clinical, echocardiographic, and angiographic findings. The poor response to medical and surgical therapy are noted.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
8/33. 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.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
9/33. Congestive cardiomyopathy in Noonan's syndrome.We report a clinicopathologic study of intractable congestive cardiomyopathy in a teenaged patient who had features of Noonan's syndrome. Various forms of congenital cardiac malformations occur commonly in Noonan's syndrome, and there are also a few reported cases of cardiomyopathy in this entity. Unlike previously documented cases, our patient was found at autopsy to have nonobstructive hypertrophic cardiomyopathy.- - - - - - - - - - ranking = 1keywords = malformation (Clic here for more details about this article) |
10/33. Isolated cardiomegaly in the second trimester as an early sign of fetal hydrops due to intracranial arteriovenous malformation.We report the case of a 27-year-old pregnant woman in whom isolated mild fetal cardiomegaly, diagnosed prenatally on sonographic examination at 22 weeks' menstrual age, was the first sign of development of an arteriovenous malformation of the vein of Galen. The arteriovenous malformation was visualized on sonographic examination at 29 weeks' menstrual age; prenatal MRI at 32 weeks confirmed the diagnosis. At 34 weeks' menstrual age, repeat sonographic examination demonstrated polyhydramnios, cardiomegaly, and generalized hydrops with ascites and pleural effusion. After vaginal delivery, the male neonate died of respiratory distress. We present the findings of prenatal gray-scale, color Doppler, and power Doppler sonography; MRI; and postmortem examination.- - - - - - - - - - ranking = 91.436631965029keywords = arteriovenous malformation, malformation (Clic here for more details about this article) |
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