1/16. 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 = vessel (Clic here for more details about this article) |
2/16. prenatal diagnosis of intrahepatic arteriovenous shunts.A case of mixed intrahepatic arteriovenous shunts in a fetus diagnosed at 35 weeks' gestation is presented. color Doppler ultrasonography in the fetal liver demonstrated complicated vascular connections fed by the hepatic arterial branches and drained into the portal and middle hepatic veins. Pulsed Doppler ultrasonography identified a high cardiac output state by the detection of increased flow velocities of the great vessels. The contribution of Doppler ultrasonography to hemodynamic changes is highlighted. The management is briefly discussed.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
3/16. Pulmonary hypertension complicating portal hypertension.We report 9 patients with pulmonary hypertension complicating portal hypertension. The cause of portal hypertension was cirrhosis in 7 patients, nodular regenerative hyperplasia of the liver in 1, and portal vein obstruction in 1. Six patients had been treated by portal-systemic shunting before the clinical onset of pulmonary hypertension. The interval between the first manifestation of portal hypertension and the recognition of pulmonary hypertension ranged from 2 to 15 years. Histologic examination in 1 of these patients revealed medial hypertrophy, concentric intimal proliferation, and plexiform lesions affecting the small pulmonary arteries. Pulmonary hypertension might result from the effect of a vasoconstrictive agent on the small pulmonary arteries or of a substance toxic to the walls of these vessels that is produced in the splanchnic territory, destroyed by the liver in normal subjects, and reaches the pulmonary arteries through portal-systemic shunts in these patients.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
4/16. Hypertrophic obstructive cardiomyopathy and coronary artery spasm.Three patients had hypertrophic obstructive cardiomyopathy and coronary artery spasm. The clinical diagnosis of hypertrophic obstructive cardiomyopathy, in all patients, was confirmed by echocardiography and angiography. Significant spasm of the right coronary artery was demonstrated in each patient by selective coronary arteriography. One patient had atherosclerotic obstructive three vessel disease, while the other two showed no evidence of any fixed organic narrowing of the coronary arteries. ST segment elevation in the inferior ECG leads was documented in two of the patients in association with coronary spasm.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
5/16. Prenatal marfan syndrome: report of one case and review of the literature.OBJECTIVES: Our objective was to describe the features of prenatal marfan syndrome. methods: Doppler fetal echocardiograms were performed. The morphology and rhythm of the fetal heart were examined sequentially. RESULTS: The case was referred because of cardiomegaly and dilated great vessels. Sequential Doppler echocardiographic evaluation led to the diagnosis of prenatal marfan syndrome. The main features are cardiomegaly, dysplastic atrioventricular valves with tricuspid regurgitation and dilated great vessels, which can be aneurysmal at their origin. The fetus died in utero at 39 weeks of gestation because of cardiac failure. Pathological study confirmed the Marfan habitus and complications. Molecular genetic study showed a de novo point mutation in exon 26 of the FBN1 gene. CONCLUSION: We report a case of prenatal marfan syndrome diagnosed by sequential evaluation of the cardiac signs, which are essential for prenatal diagnosis. The prognosis seems as poor as the neonatal one. The prenatal diagnosis is essential for adequate counselling.- - - - - - - - - - ranking = 2keywords = vessel (Clic here for more details about this article) |
6/16. Pulmonary hypertension in connective tissue disease. Report of three cases and review of the literature.patients with connective tissue disease (CTD) who are prone to developed isolated pulmonary hypertension (PH) are primarily young females with a history of Raynaud's phenomenon associated with an exertional dyspnoea. From the start of the disease, pulmonary function tests show a decreased diffusing capacity for carbon monoxide, while X-ray examination shows no obvious abnormalities such as interstitial fibrosis. All patients show, on electrocardiographic examination, evidence of right axis deviation and right ventricular hypertrophy. It has been suggested that PH is found mostly in patients with systemic scerlosis characterized by the crest syndrome. The histopathological findings are intimal proliferation, narrowing of the vessel lumen and medial fibrosis. These are not specific for CTD. One would expect more signs of vasculitis. Neither signs of lung fibrosis, nor signs of pulmonary emboli are described.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
7/16. Total anomalous pulmonary venous drainage and sudden death in infancy.The clinicopathologic features of four infants with undiagnosed total anomalous pulmonary venous return who died suddenly and unexpectedly are presented. Two infants were found dead in their beds and two collapsed following very non-specific episodes of mild tachypnoea and tachycardia, respectively. Pulmonary venous blood drained via a common channel to the left innominate vein, the superior vena cava, the inferior vena cava at the junction of the hepatic vein and the supradiaphragmatic portion of the inferior vena cava. All cases demonstrated right atrial and ventricular hypertrophy and in one case there was associated hypoplastic left heart syndrome. Although those mechanisms responsible for sudden infant death syndrome could not definitely be excluded in two cases, the presence of anomalous pulmonary drainage with significant right-sided cardiac cardiac hypertrophy in each patient strongly suggests a contributing role for the vascular malformation in the aetiology of sudden unexpected death. The importance of careful in situ dissection of the major vessels in all cases of paediatric sudden death is emphasised, particularly in the presence of other congenital cardiac anomalies.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
8/16. Unilateral megalencephaly associated with neonatal high output cardiac failure.High-flow cerebral arteriovenous fistulae are well known to present in the neonatal period with macrocephaly, cranial bruits and high-output cardiac failure. This report describes a newborn infant with such a clinical presentation, who had unilateral megalencephaly without macroscopic arteriovenous shunts. Ultrasound Doppler examination of the carotid and cerebral vessels showed diastolic flow, or a decreased pulsatility index, consistent with decreased intracranial vascular resistance. The ipsilateral cerebral arteries and veins were markedly enlarged at angiography and at post-mortem examination, but there was no arteriovenous malformation. The infant expired from high-output cardiac failure and hypoglycemia. It is postulated that the high-output cardiac failure was due to increased blood flow through the enlarged, dysplastic cerebral hemisphere.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
9/16. Pulmonary blood vessels and endocrine cells in subacute infantile mountain sickness.A male infant of 16 months, of the Han race, died from subacute infantile mountain sickness in Lhasa (3600 m). At necropsy there was right ventricular hypertrophy secondary to muscularization of the pulmonary arteries and arterioles thought to have been induced by hypobaric hypoxia. In addition, there was intimal proliferation of myofibroblasts in the pulmonary arterioles, venules and veins. There were increased numbers within the bronchioles of pulmonary endocrine cells, containing calcitonin and bombesin, which could be related to hypoxia or trophic effects on the pulmonary vasculature. The relation of delayed effects of hypoxia to primary pulmonary hypertension is considered in this study.- - - - - - - - - - ranking = 4keywords = vessel (Clic here for more details about this article) |
10/16. Anomalous origin of the left anterior descending coronary artery from the pulmonary trunk: recognition in life and successful surgical treatment.An anomalous origin of the left anterior descending coronary artery from the pulmonary trunk with the right and left circumflex arteries arising from the aorta is very rare and the diagnosis made only at necropsy. An anomalous coronary artery was suspected owing to unexplained cardiomegaly in a 17 month old girl and the anatomy was defined by angiography. The anomalous vessel was successfully reimplanted into the aorta.- - - - - - - - - - ranking = 1keywords = vessel (Clic here for more details about this article) |
| | Next -> |