Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm with herniation of abdominal viscera into the thorax. The morbidity and mortality in cases is caused primarily by pulmonary hypoplasia. A prenatal diagnosis of CDH can be established by ultrasound, but ultrasound cannot detect pulmonary hypoplasia with certainty. We evaluated pulmonary hypoplasia by the simple method of lung intensity. The subjects were eight fetuses. In four fetuses, the lungs showed low intensity, and these fetuses all had pulmonary hypoplasia. Two of the four fetuses with high intensity showed a good prognosis, however, the other two fetuses did not. One of the two cases with poor prognosis had complex congenital heart disease, and the other died of sepsis and pulmonary hypertension. A high signal did not always promise a favorable prognosis, probably because accompanying abnormalities dominated the prognosis. MR assessment of lung intensity in fetuses with CDH may be useful in evaluating the severity of pulmonary hypoplasia. Low signal of the lung may reflect pulmonary hypoplasia and suggests a poor prognosis.
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PMID:[Evaluation of pulmonary hypoplasia with congenital diaphragmatic hernia]. 1592 Sep 69

Nonimmune hydrops fetalis (NIHF) or generalized soft tissue edema and cavity effusions may be due to cardiovascular diseases, congenital infections, genitourinary malformations, thoracic masses, placental conditions, chromosomal abnormalities, and idiopathic. We report 32 cases of NIHF from among 429 neonates who underwent autopsies (incidence 7.45%). Sixteen cases (50%) had cardiovascular disease; all were due to low output cardiac failure; 7 had structural congenital heart disease. Three of the children with congenital heart disease also had chromosomal abnormalities: 2 had trisomy 18 and 1 had Noonan syndrome. Among myocardial conditions were five subjects with cardiomyopathies (1 of each of the following types): oncocytic, dilated, endocardial fibroelastosis, cardiac glycogenosis, and carnitine deficiency; 3 had myocarditis, and 1 had cardiac rhabdomyomas. Congenital infections were due to cytomegalovirus in 3 cases, bacteria in 2, and parvovirus in 1. The mechanism of NIHF in these cases might be a combination of decreased myocardial contractility due to myocarditis and fetal anemia. Genitourinary diseases were present in 5 newborns: Two had congenital nephrotic syndrome, 1 had VACTER association, 1 had prune-belly syndrome, and 1 had urogenital sinus malformation. Intrathoracic lesions were found in 2 babies (pulmonary sequestration and diaphragmatic hernia). One twin died of volume overload due to twin transfusion syndrome. Only 2 newborns were classified as idiopathic. Our study shows that cardiovascular diseases that lead to heart failure or impaired venous return are more common in the liveborn (50%), whereas congenital infections are more common in the stillborn with NIHF.
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PMID:Nonimmune hydrops fetalis in the liveborn: series of 32 autopsies. 1601 Apr 81

We report on a 3-year-old girl with psychomotor retardation, cardiopathy, strabismus, umbilical hernia, and facial dysmorphism in whom a de novo unbalanced submicroscopic translocation (10p;18q) was found by MLPA (Multiplex Ligation dependent Probe Amplification) and FISH analyses. Additional FISH studies with locus specific RP11 BAC probes and analyses with microsatellites revealed that the translocation resulted in a deletion estimated between 6 and 9 Mb on the maternal chromosome 18 and a subtelomeric 10p duplication of approximately 6.9 Mb. The proband's karyotype is 46,XX.ish der(18) t(10;18)(18pter-->18q23:10p15 --> 10pter). A subterminal duplication of 10p, as well as a subterminal deletion of 18q have been rarely reported so far. The clinical phenotype of this patient is reviewed and discussed.
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PMID:A de novo subterminal trisomy 10p and monosomy 18q in a girl with MCA/MR: case report and review. 1648

A 4-month-old infant with trisomy 21 is brought to the emergency department for breathing difficulty. Subsequently, he is diagnosed with a Morgagni diaphragmatic hernia. Tachypnea in an infant with trisomy 21 may be the result of diagnosis such as congenital heart disease, infectious processes, structural anomalies, or aspiration secondary to gastroesophageal reflux or swallowing dysfunction. Knowledge of these abnormalities is important when generating a differential diagnosis in these patients. Although uncommon, a Morgagni hernia may present beyond the newborn period and should be considered when evaluating an infant with trisomy 21 and respiratory difficulty.
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PMID:An infant with trisomy 21 and tachypnea. 1662

A case of right diaphragmatic hernia is presented associated with a complex congenital heart disease (double-outlet right ventricle, transposition of the great arteries and left isomerism) diagnosed prenatally. Despite high-frequency oscillatory ventilation plus nitric oxide and uneventful repair of the hernia, the infant died after 6 days of uncontrolled pulmonary hypertension and severe aortic coarctation that developed postnatally.
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PMID:Right congenital diaphragmatic hernia associated with a complex heart disease. 1685 46

The vertebral heart size (VHS) is used to objectively assess cardiac dimensions on thoracic radiographs. A high VHS suggest the presence of cardiac pathology, such as dilated cardiomyopathy, degenerative atrioventricular valvular disease, pericardial effusion, pericardioperitoneal diaphragmatic hernia, tricuspid dysplasia, ventricular septal defect, or patent ductus arteriosus, among others. However, breed or body conformation can influence the VHS. Because Greyhounds have a high prevalence of physiologic systolic murmurs associated with high aortic velocity, and large cardiac dimensions when compared with dogs of similar size, they are frequently suspected of having heart disease. The purpose of this study was to compare the VHS in normal Greyhounds with those in Rottweilers, and a group of dogs from various other breeds using both analog and digital radiology. The VHS was significantly higher in Greyhounds (P< 0.0001), when compared with Rottweilers and to other dog breeds. The mean VHS on lateral radiographs for Greyhounds was 10.5 +/- 0.1, for Rottweilers it was 9.8 +/- 0.1, and for mixed breed dogs it was 10.1 +/- 0.2. This study confirms that the relative cardiomegaly reported in necropsy and echocardiographic studies in Greyhounds is easily detected using plain radiography and the VHS.
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PMID:Vertebral heart size in retired racing Greyhounds. 1769 32

Cardiac malposition is a rare but important finding when detected on fetal ultrasound. The purpose of this study was to evaluate the incidence of fetal cardiac malposition, associated abnormalities, and clinical outcome in a tertiary-care medical center. Records of fetuses (1993 to 2006) with dextroposition, dextrocardia, mesocardia, ectopia cordis, or heterotaxy were reviewed. The presence of congenital heart disease (CHD), extracardiac anomalies, and outcome were noted. Cardiac malposition was present in 101 fetuses among a total of 3313 (3%) pregnancies. In 78 (78%) patients, the heart was positioned in the right hemithorax. Of those, 26 (33%) had dextrocardia (CHD = 21), and 52 (67%) had dextroposition (CHD = 14). Sixteen (16%) patients had mesocardia (CHD = 8), and 7 (7%) had ectopia cordis (CHD = 6). The majority (58%) of fetal cardiac malposition was caused by intrathoracic masses. Concomitant CHD occurred in 50%. Outcome was available in 97%. The perinatal and neonatal mortality rate was 30%; the elective termination rate was 4%. Patients with CHD had higher mortality rates. The highest mortality rates occurred in ectopia cordis and combined disease of diaphragmatic hernia and CHD.
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PMID:Fetal cardiac malposition: incidence and outcome of associated cardiac and extracardiac malformations. 1840 41

Jarcho-Levin syndrome (spondylothoracic or spondylocostal dysostosis) is an eponym that is used to define individuals with a short neck, short trunk, and short stature and multiple vertebral anomalies. The prognosis is directly related to respiratory complications. Reported findings associated with Jarcho-Levin syndrome include congenital heart defects, abdominal wall malformations, genitourinary malformations, upper limb anomalies, and neural tube defects. We report on a 6-day-old girl who presented with an incomplete form of Jarcho-Levin syndrome with late-presenting congenital diaphragmatic hernia and congenital heart disease.
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PMID:Jarcho-Levin syndrome presenting with diaphragmatic hernia. 1862 69

Laparoscopic Roux-en-Y gastric bypass (LGB) is one of the most popular surgeries for morbid obesity. Robotic use is also on the rise. Data concerning outcomes is limited, hence the need for more information. The first 100 robotic-assisted bypasses by one surgeon in one institution were studied. Data obtained from clinic notes and hospital records included all who underwent the procedure. There were 79 females and 21 males. Mean age and body mass index were 42 years and 48 kg/m2, respectively. Comorbidities included diabetes, 22 per cent; hypertension, 47 per cent, gastroesophageal reflux disease, 40 per cent; obstructive sleep apnea, 53 per cent; dyslipidemia, 17 per cent; and heart disease, 8 per cent. Prior surgeries included cesarean -section, 26 per cent; cholecystectomy, 17 per cent; hysterectomy, 3 per cent; hernia, 1 per cent, and other abdominal surgery, 27 per cent. Intraoperatively procedures included adhesiolysis, 22 per cent; cholecystectomy, 16 per cent; and herniorrhaphy, 3 per cent. Average time was 177.7 minutes. Mean stay was 1.51 days. Thirty-day mortality was 0. Emergency department re-evaluations included 13. Most were minor problems. There was one gastrojejunal leak. Early complications included leak, thrombosis, and bleeding requiring transfusion in four patients. There were four strictures. Overall follow up was greater than 90 per cent. Average weight loss was 21.2 per cent of excess body weight by Month 1, 33.8 per cent by Month 3, and 50.7 per cent by Month 6. Learning curves for time and major complications were 30 and 50 cases, respectively (P = 0.03, 0.04). Robotic use in bariatrics is possible in community hospitals. Although technologies are still in their infancy, complication rates and weight loss are comparable to nonrobotic procedures.
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PMID:100 robotic-assisted laparoscopic gastric bypasses at a community hospital. 1894 36

Nitric oxide, a gas molecule, is a unique pharmaceutical agent that can be inhaled and thus delivered directly to the lung. More than a decade of intensive laboratory and clinical investigation has culminated in the current role for inhaled NO as the only selective pulmonary vasodilator for the treatment of persistent pulmonary hypertension of the newborn (PPHN). Not surprisingly, this potent and successful therapy continues to be studied intensively to better define its mechanism of action and role in PPHN treatment. In addition, there remains intense interest in possible new applications for newborns, as well as strategies that may enhance its efficacy. This review describes several areas of current research on amplification of NO signaling in the neonatal pulmonary vasculature, and reviews our current knowledge about the role of iNO in other conditions such as congenital diaphragmatic hernia and congenital heart disease. In addition, laboratory and clinical studies addressing a potential role for iNO as a therapeutic modality for the preterm infant are reviewed.
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PMID:Inhaled NO in the experimental setting. 1894 45


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