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Query: UMLS:C0018799 (heart disease)
 34,133 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Once the rules for understanding of complex congenital heart disease were reviewed in part I, the purpose in this second part is show how the clinical diagnosis is made. The first requirement is to separate patients into two groups: neonates and infants. A critical route based on five clue data: patient's age, presence or absence of cyanosis, presence or absence of myocardial failure, pulmonary blood flow estimated on x chest film, and presence of either right or left ventricular hypertrophy or both in ECG, is matched. This approach is helpful to reduce possibilities to diagnosis in congenital heart disease.
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PMID:[Understanding and diagnosis of complex congenital cardiopathies. II]. 141 55

Persistence of the fetal circulation (PFC) is a syndrome characterized by failure of the cardiocirculatory system to adapt successfully to postnatal life. Its typical feature is persisting right-left shunt across fetal channels which determines cyanosis refractory to oxygen treatment. PFC can simulate cyanotic congenital cardiopathy. It has two forms: a primitive form and secondary one due to various causes especially perinatal asphyxia. Both forms have a common pathogenesis consisting of hypertension of the pulmonary arterial circulation. This article reviews the physiology of the main prenatal and postnatal circulatory characteristics and the factors which regulate the pulmonary circulation. It also reports the latest findings on PFC physiopathology and treatment indicating the prognostic factors and future perspectives.
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PMID:[Physiopathologic and therapeutic aspects of persistent fetal circulation. Review of the literature and personal histological observations]. 146 77

To evaluate the effect of chronic hypoxemia on brainstem maturation, auditory brainstem responses were examined in 70 children (32 with and 38 without cyanosis) who had congenital heart disease. Ninety-one age-matched normal children served as controls. At 1-3 months of age, the I-V interpeak latencies of cyanotic infants (mean +/- S.D.; 5.17 +/- 0.17 ms) were more prolonged than were those of controls (4.95 +/- 0.11 ms) and those without cyanosis (4.84 +/- 0.22 ms; P < .05; P < .01). At 4-11 months of age, the I-V interpeak latencies of cyanotic infants (4.85 +/- 0.13 ms) were more prolonged than were those of controls (4.67 +/- 0.19 ms) and those not experiencing cyanosis (4.5 +/- 0.17 ms; P < .05; P < .01). In the cyanotic children, there was a significant negative correlation between the I-V interpeak latency and oxygen partial pressure (P < .01) or oxygen saturation (P < .05). Three of the 70 patients (4.3%) with congenital heart disease had absent auditory brainstem response. These data indicate that chronic hypoxemia may be one of the factors in retarded brainstem maturation.
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PMID:Auditory brainstem responses in congenital heart disease. 147 72

A number of practical office and bedside clues to cardiac disease in infants and children have been passed on through the years. They relate to the history, to the inspection and palpation components of the physical examination, and to knowledge of the specific cardiac defects that are likely to be associated with certain clinical syndromes. With the possible exception of coarctation of the aorta, the clues are not diagnostically specific. In many instances, however, they serve to narrow a broad array of diagnostic possibilities to 2 or 3 and, with the aid of other clues and auscultation, they can often be distinguished from one another. When a primary care physician is confronted with a child who has an incidental murmur that is "probably" innocent but could be organic, useful clues favoring an organic murmur are a history of congenital heart disease in a first-degree relative; a history of maternal rubella syndrome, alcohol use, or teratogenic drug use during pregnancy; a history of inappropriate sweating; a history of syncope, chest pain, or squatting; maternal diabetes mellitus; premature birth; birth at a high altitude; cyanosis; abnormal pulsations; recurrent bronchiolitis or pneumonia; chronic unexplained hoarseness; asymmetric facies with crying; and a physical appearance suggestive of a clinical syndrome.
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PMID:Clues in diagnosing congenital heart disease. 157 99

Chest closure after cardiac surgery occasionally results in cardiac compression leading to circulatory failure. In shunt-dependent circulation, the arterial oxygen saturation may decrease significantly due to the increase in pulmonary vascular resistance caused by chest closure. Temporary patch implantation with delayed sternal closure facilitates circulatory and/or pulmonary stabilization (temporary chest wall patch plasty, TCWPP). Between July 1986 and June 1991, 42 patients underwent staged chest closure (TCWPP) after open heart surgery for congenital lesions (4.9% of 854 patients). TCWPP was performed when either primary hemodynamic deterioration or an increase in cyanosis (palliative procedures only) followed by hemodynamic deterioration occurred during attempted or shortly after sternal closure. Overall mortality was 40.4% (17/42). It was 32.3% (11/34) when the patch was inserted primarily at the end of the operation. If the patch was inserted emergently 4-24 h postoperatively, mortality was 75% (6/8). Definite chest closure was performed from 4 h to 6 days (mean 72 h) postoperatively. In 2 patients closure had to be performed emergently (single ventricles); 7 patients died before chest closure. One mediastinal microbiology examination was positive. Deep sternal infection necessitating operative revision occurred in one other patient. In conclusion, TCWPP may considerably lower mortality of the illest patients after surgery for complex congenital heart disease. A timely decision as to the performance of staged chest closure is mandatory. This procedure rarely causes infection. We now apply this technique liberally, by cardio-mediastinal size judgement in over 30% of our TCWPP candidates even without a prior trial of primary closure.
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PMID:Staged chest closure in pediatric cardiac surgery preventing typical and atypical cardiac tamponade. 158 Oct 87

Pulmonary artery banding is indicated in numerous congenital cardiac defects not amenable to a total repair. One complication of pulmonary artery banding, especially in neonates who require early banding, is progressive cyanosis during the rapid growth phase requiring earlier than anticipated total correction, which may produce a less than optimal result. A simple pulmonary artery band that enlarges as the patient grows would avoid this early complication. We report a technique of pulmonary artery banding in an animal model using different absorbable sutures. The band enlarges in a prescribed staged fashion without any further intervention, hopefully allowing growth of neonates and infants with complex congenital heart disease.
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PMID:A staged expanding pulmonary artery band. 843 Oct 89

We present a case of the rare coincidence of three mechanisms leading to development of congenital heart disease in intrauterine life: intrinsic defect of the development of the cardiac loop (dextrocardia), failure of normal expansion of the subpulmonary infundibulum (Fallot syndrome) and endocardial cushion defect (common atrium and common atrioventricular valve ). It was associated with partial viscera inversion. A 31-year old man with congenital cyanotic heart disease, and Blalock-Taussig anastomosis was admitted to the hospital due to symptoms of severe cardiac failure. On physical examination: systolic murmur, hepatomegaly, ascites, leg's edema and cyanosis were found. In ECG--atrial fibrillation with 3-d degree a-v block. Standard echocardiography revealed: dextrocardia, a large single atrium with ostia of pulmonary and systemic veins, single atrio-ventricular valve , large ventricular, Fallot-like septal defect. The papillary muscles were not visible in the left ventricle. Aorta and pulmonary trunk arose from morphological right ventricle. The patient died on the 3-rd day of hospitalization in the course of cardiac and respiratory insufficiency. Postmorten examination confirmed the diagnosis.
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PMID:[Combined heart defects: tetralogy of Fallot, common atrium and a single atrioventricular valve diagnosed by echocardiography]. 164 Jun 69

In this second part of our review, the diagnosis of the following congenital heart disease is discussed: aortic stenosis, aortic isthmus stenosis (coarctation of the aorta), Fallot's tetralogy and transposition of the great vessels. Aortic stenosis and coarctation of the aorta each represents a spectrum of cardiac diseases of varying severity. Cases that are clinical less severe may escape diagnosis until late childhood or adolescence. Fallot's tetralogy and transposition of the great vessels in contrast, lead to cyanosis, and are therefore usually diagnosed already in the young infant. In all four conditions, the suspected diagnosis can be established on the basis of clinical or auscultatory findings. Further diagnostic clarification is achieved with the aid of non-invasive procedures such as CT scan, chest X-ray, echocardiography and, where indicated, NMR imaging. Additional cardiac catheterization is required only in the case of the tetralogy of Fallot.
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PMID:[Diagnosis of congenital heart defects today. Part 2: Aortic stenosis, aortic isthmus stenosis, tetralogy of Fallot, transposition of great vessels]. 164 89

The relation between pulmonary vascular pathology and hemodynamics in 81 patients with congenital heart disease complicated by pulmonary hypertension was studied. Slight to marked increase of mean pulmonary pressure (MPAP) was seen in 5 of them, but no vascular lesion was found. 59 of the 81 cases had marked increase of MPAP and Grade 1-2 pulmonary vascular lesions, and cyanosis was seen clinically. Postoperative effects were satisfactory, indicating that not all patients with cyanosis contraindicated surgical treatment. The rest (17) of the 81 patients had most marked increase of MPAP and Grade 3-4 pulmonary vascular lesions with good postoperative results, but long-term results await follow-up observations. It is thought that Grade 4 pulmonary vascular lesion should be considered as a contraindication for corrective surgery.
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PMID:[Comparison between pulmonary vascular pathology and hemodynamics in congenital heart disease with pulmonary hypertension]. 164 27

Tetralogy of Fallot is the most common malformation of children born with cyanotic heart disease, with an incidence of approximately 10 per cent of congenital heart disease. There can be a wide spectrum as to the severity of the anatomic defects, which include ventricular septal defect, aortic override, right ventricular outflow tract obstruction, and right ventricular hypertrophy. Cyanosis may vary from mild to severe, and patients may present as newborns or, more commonly, young infants. Infants with classic tetralogy of Fallot and stable anatomy should undergo primary complete intracardiac repair. The overall hospital mortality is approximately 3 to 5 per cent, with most patients who survive having an excellent clinical and hemodynamic result.
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PMID:Tetralogy of Fallot. 168 16


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