Cases reported "Heart Septal Defects"

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1/214. Bedside cardiac catheterization using transesophageal echocardiographic guidance.

    We describe for the first time the use of transesophageal echocardiography to guide cardiac catheterization performed at the bedside in the pediatric cardiac intensive care unit. The procedure was performed on a 2.6-kg infant with ventricular and atrial septal defects after repair of coarctation of the aorta. Poor hemodynamic status prevented obtaining the required hemodynamic information from cardiac catheterization in the cardiac catheterization laboratory. Transesophageal echocardiography provided cardiac and vascular imaging, which helped guide catheter placement where the small size of the infant and the extensive thoracic postoperative bandages prevented obtaining the echocardiographic information from other windows. ( info)

2/214. The physiology of congenital heart disease: assessment by Doppler color flow mapping.

    The use of Doppler echocardiography is a routine part of the noninvasive assessment of the patient with heart disease. In children with congenital heart disease, pulsed- and continuous-wave Doppler echocardiographic techniques provide accurate, reproducible hemodynamic data relative to structural defects. Doppler color flow imaging, however, allows for qualitative assessment of blood flow patterns, which may give important insights into the changing physiology of the newborn infant or that of a patient in the medical or surgical intensive care settings. Ten cases are presented in which this flow information is instructive in understanding the physiological sequelae of congenital heart disease. ( info)

3/214. An isolated ventricular septal defect as a consequence of penetrating injury to the heart.

    The authors describe, in a case report, an isolated defect of the ventricular septum developing due to a stab injury to the heart not requiring an emergency surgical intervention. Two months after the injury, the authors performed primary surgical correction of the defect. ( info)

4/214. Septal cystic mass in the right atrium: echocardiographic evaluation.

    Right atrial masses rarely are seen in children and are usually benign. Clinical manifestations mainly depend on the location of the mass and whether it alters blood flow. This report describes the clinical course, echocardiographic findings, pathology, and serial follow-up of an unusual cystic mass with a mixture of tissue common to the atrioventricular septum located in the posteroinferior right atrium. ( info)

5/214. Circumscribed intestinal protein loss with deficiency in CD4 lymphocytes after the fontan procedure.

    Protein-losing enteropathy is an important complication after right heart bypass operations (fontan procedure). Laboratory examinations usually reveal hypoalbuminaemia, hypoproteinaemia, elevated alpha(1)-antitrypsin clearance, and lymphocytopenia. A case of protein-losing enteropathy after fontan procedure is reported with a circumscribed protein loss in the region of the terminal ileum despite good haemodynamics. The patient developed only mild hypoalbuminaemia and no diarrhoea but severe cellular and humoral immune abnormalities, namely a markedly decreased proportion of CD4 lymphocytes but normal proportion of CD8 lymphocytes (CD4 14%, CD8 23%) and decreased serum levels of immunoglobulin g. Intestinal biopsies revealed normal mucosa. This report is unique as it is the first to describe a ratio of CD4 to CD8 lymphocytes <1 due to an almost selective loss of CD4 lymphocytes and a circumscribed intestinal protein loss in a patient who developed protein-losing enteropathy after Fontan operation. CONCLUSION: There is a severe decrease of CD4 lymphocytes of unknown origin in a patient with circumscribed intestinal protein loss after Fontan operation. Passive leakage of lymph fluid due to abnormal systemic venous pressure is not a sufficient explanation of the almost selective loss of CD4 lymphocytes. Primary or secondary activation of the immune system may influence structural integrity and permeability of the intestinal wall and may play a triggering role in protein-losing enteropathy after the fontan procedure. ( info)

6/214. Common arterial trunk associated with atrioventricular septal defect.

    Described are the clinical, cross-sectional echocardiographic, electrocardiographic and angiographic findings, together with the results at autopsy, in a 5-month old infant with a common arterial trunk associated with an atrioventricular septal defect. As far as is known, this is only the 13th such case to be reported in the literature. A particularly unusual feature of this case was an intact atrial component of the atrioventricular septal defect. ( info)

7/214. A new, safe, and easy technique of atrial septal defect creation.

    We created an atrial septal defect (ASD) using a Ferris-Smith-Kerrison bone punch under transesophageal echocardiographic monitoring for infants with complex congenital heart diseases, eg, transposition of the great arteries. We describe a safe and easy technique of ASD creation instead of Blalock-Hanlon operation. ( info)

8/214. Coexistence of a left posteroseptal tract with persistent left superior vena cava. Ablation through an anomalous superior vena cava.

    A case of a young male with WPW syndrome due to a left posteroseptal tract associated with a persistent left superior vena cava is described. After unsuccessful ablation attempts with a number of different approaches at conventional target sites, the accessory connection was successfully ablated within the coronary sinus. This was achieved only when the ablation catheter was introduced through the persistent left superior vena cava. ( info)

9/214. Premature closure of foramen ovale and renal vein thrombosis in a stillborn twin homozygous for methylene tetrahydrofolate reductase gene polymorphism: a clinicopathologic case study.

    Premature closure of the foramen ovale, 4-chamber cardiac hypertrophy, and renal vein/vena cava thrombosis were found at autopsy of a stillborn dizygotic twin at 36 weeks gestational age. review of the original prenatal sonograms showed features suggestive of early closure of the foramen ovale. Homozygosity for the 5, 10 methylene tetrahydrofolate reductase mutation was shown only in the affected twin after the parents were found to be heterozygous for the mutation. The difference in outcome of the twins following prenatal treatment with beta mimetics and corticosteroids for preterm labor may be related to the added susceptibility factor for thromboembolism associated with presumed hyperhomocysteinemia in the proband which was not shared by the surviving healthy twin. The role of premature closure of the foramen ovale and prenatal treatment are discussed but remain uncertain. ( info)

10/214. Familial recurrence of nonsyndromic interrupted aortic arch and truncus arteriosus with atrioventricular canal.

    BACKGROUND: multifactorial inheritance is probably involved in most cases of nonsyndromic conotruncal heart defects (CHDs), but Mendelian transmission is often suspected. RESULTS: We report on a family with recurrence of nonsyndromic CTHD in two double first cousins; i.e., two brothers married two sisters. One of the cousins (case 1) had interrupted aortic arch (IAA) type B, while the other one (case 2) had truncus arteriosus (TA) with atrioventricular canal defect (AVCD). CONCLUSIONS: Our family further supports monogenic inheritance of CTHDs. In addition, the presence of TA associated with AVCD in one of the patients confirms the higher occurrence of CTHD in families with complex TA. The absence of 22q11 microdeletion (del22q11) in our patients, as in several literature reports of familial CTHDs, supports the existence of genes different to those located on chromosome 22q11 which could be implicated in the pathogenesis of CTHDs. ( info)
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