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PubMed2005

Nutrition care for newborns with congenital heart disease.

Those health care professionals entrusted with the care of infants with congenital heart disease require an understanding of the unique nutritional needs of this population. This article defines the congenital, physiologic, and nutritional variables encountered in this population. The nutritional needs, multi-factorial sources of undernutrition, and consequences of inadequate nutrition in infants with congenital heart disease are discussed, as well as medical and nutritional management strategies intended to optimize growth and reduce morbidity.

Clinical Trials as Topic↗
PubMed1990

Airway obstructions associated with congenital heart disease in infancy.

Significant airway obstruction may complicate the natural and surgical history of infants with congenital heart diseases. Airway obstruction occurred in 12 infants who had operations for congenital heart disease. In all cases tracheography demonstrated the cause of airway obstruction. Significant mortality (5/12, 41.7%) and morbidity in this group of infants were the result of airway obstruction. To reduce the complications caused by airway obstruction in infants with congenital heart disease, we recommend (1) preoperative identification of patients with potential airway obstruction, (2) preoperative tracheography in high-risk infants, (3) appropriate choice of the surgical procedure, especially when insertion of a prosthetic conduit is required, (4) early recognition of the problem during the postoperative period, (5) prompt diagnosis by postoperative tracheography, with or without angiography, and (6) therapy directed at the cause of airway obstruction.

Airway Obstruction↗
PubMed1997

The haematological management of patients with cyanotic congenital heart disease. A time for consensus?

AIMS: Recurrent venesection of patients with cyanotic congenital heart disease may be detrimental, with an increased risk of cerebrovascular events and symptomatic iron-deficiency. The aim of this study was to determine the venesection policies as practised in hospitals within a U.K. region and to determine if these policies followed current recommendations. METHODS AND RESULTS: Fifty-eight consultants (56% response rate) in cardiac specialties completed self-assessment questionnaires regarding the indications for and practice of venesection. Sixty-one percent of those responding were involved directly in the care of patients with cyanotic congenital heart disease and of these clinicians 97% used venesection. Indications for venesection varied, with 51% of those responding using an elevated haemoglobin per se (6.5-21.0 g. dl-1); 78% an elevated haematocrit (0.55-0.75) and 83% symptoms. Desired maintenance haemoglobin and haematocrit levels also varied greatly. Fifty percent of the consultants responding routinely screened their patients for iron deficiency and 23% felt there was no indication for investigating a low mean corpuscular volume. Only 18% of the policies described followed any evidence based principles. CONCLUSIONS: The practice of venesecting patients with congenital cyanotic heart disease varies greatly. Policies in many hospitals do not reflect the minimal benefits and considerable risks associated with recurrent venesection.

Cyanosis↗
PubMed1990

Magnetic resonance imaging of congenital heart disease.

MRI has assumed an important role in the noninvasive evaluation of patients with congenital cardiac lesions. In this article, the authors examine basic technical considerations, an approach to analysis of MR images in congenital heart disease patients, and review major applications of MRI as they apply to patients with congenital heart disease.

Heart Defects, Congenital↗
PubMed1984

Congenital heart disease in adults.

From January 1972 to April 1982, 125 adults underwent operation for congenital heart disease at the Medical University of South Carolina. Age ranged from 18 to 73 years, with an average age of 31 years. A variety of defects were seen. Atrial septal defect (59 patients), ventricular septal defect (15 patients), patent ductus arteriosus (14 patients), tetralogy of Fallot (14 patients), and coarctation of the aorta (9 patients) were the most common. There were 48 men and 77 women in the study. Most patients presented with recent onset of dyspnea on exertion or at rest, but 16 patients were asymptomatic. Eight patients died after surgery for an overall operative mortality rate of 6 per cent. The most common postoperative complication was arrhythmia (13 patients, 10%). Despite recent advances in the diagnosis and treatment of congenital heart disease in infants, a significant number of patients apparently escape detection and first present with congenital heart disease as adults. Prompt diagnosis and surgical intervention will usually produce excellent results and relief of symptoms in these patients.

Adolescent↗
PubMed1977

[Alterations of pulmonary vessels in congenital heart disease. A summarized differential diagnostic table (author's transl)].

Roentgen signs of congenital heart disease are demonstrated by plain p. a. chest films and kymograms. Diagnostic critieria are discussed which cause increased, decreased, or minimal altered pulmonary blood flow. This simple diagnostic method in combination with the clinical symptom of cyanosis allows differentiation of congenital heart disease prior to catheter examination.

Cyanosis↗
PubMed2000

Rapid cardiopulmonary support for children with complex congenital heart disease.

BACKGROUND: Extracorporeal membrane oxygenation has limitations in children with congenital heart disease (prolonged setup times, increased postoperative blood loss, and difficulty during transport). We developed a miniaturized cardiopulmonary support circuit to address these limitations. PATIENTS AND METHODS: The cardiopulmonary support system includes a preassembled, completely heparin-coated circuit, a BP-50 Bio-Medicus centrifugal pump, a Minimax plus membrane oxygenator, a Bio-Medicus flow probe, and a Bio-trend hematocrit/oxygen saturation monitor. Short tubing length permits a 250-mL bloodless prime in less than 5 minutes. From 1995 to 1997, 23 children with congenital heart disease were supported with this technique. RESULTS: Overall survival to discharge was 48% (11 of 23 patients). Survival to discharge was 80% (4 of 5) in the preoperative support group, 20% (1 of 5) in the postoperative failure to wean from cardiopulmonary bypass group, 44% (4 of 9) in the group placed on support postoperatively after transfer to the intensive care unit, and 50% (2 of 4 patients) in the nonoperative group. Neonatal cardiopulmonary support survival to discharge was 46% (6 of 13 patients). CONCLUSIONS: This pediatric cardiopulmonary support system is safe and effective. Advantages over conventional extracorporeal membrane oxygenation include rapid setup time, decreased postoperative blood loss, and simplified transport.

Blood Transfusion↗
PubMed2000

Extremely thickened media of small pulmonary arteries in fatal pulmonary hypertension with congenital heart disease--a morphometric and clinicopathological study.

There are patients with congenital heart disease and fatal pulmonary hypertension in whom the medial hypertrophy of the small pulmonary arteries is quite beyond the extent of ordinary cases of hypertension, a condition described as pulmonary hypertension with extremely thickened media of small pulmonary arteries (PH/ETM). Lungs from 6 infants, all younger than 2 years of age, who had congenital heart disease and fatal pulmonary hypertension, were analyzed by accurately measuring the media using Suwa's method. In PH/ETM, the media of the small pulmonary arteries was shown to be not only unusually thick, but extending toward the periphery, whereas the intimal changes were unexpectedly mild. In the PH/ETM group, the % wall thickness at a diameter of 50 microm (%Tw(50)), determined from regression analysis, was 23.2+/-1.3%, which was significantly higher than in either the control (10.3+/-1.2%) or ventricular septal defect group (18.9+/-1.6%). In persistent pulmonary hypertension of the newborn (PPHN), it was 22.3+/-1.8%, not significantly different from PH/ETM. The striking medial hypertrophy in PH/ETM and PPHN was apparently confined to small pulmonary arteries and in both conditions is likely to be the result of maldevelopment of these arteries. Surgical intervention may trigger a critical elevation of the pulmonary arterial resistance.

Autopsy↗
PubMed1999

[Clinical significance of cardiac axis determination in prenatal echocardiographic diagnosis of fetal congenital heart disease].

OBJECTIVE: To investigate the clinical significance of cardiac axis determined by echocardiography in prenatal diagnosis of fetal congenital heart disease (CHD). METHODS: 518 high risk fetuses for CHD were and 90 normal fetuses were examined by echocardiography. Cardiac axis was measured on four-chamber view and fetal cardiac anatomical structure was examined in multiple views to determine CHD. RESULTS: The 35 fetuses with CHD diagnosed by prenatal echocardiography. There is significant difference of caidiac axis between CHD group (58.1 +/- 15.7 degree) and normal group (44.8 +/- 10.3 degree, P < 0.01). Sensitivity of CHD diagnosis using four-chamber view alone is 68.5%, it will be elevated to 91.4% by combination of four-chamber view and cardiac axis determination (P < 0.05). CONCLUSION: Cardiac axis play an important role in fetal echocardiographic screening for CHD and it should be measured routinely as part of the four-chamber view of the fetal heart. The normal range of fetal cardiac axis is 20 to 75 degree. If it was more than 75 degree, the CHD should be suspected.

Adult↗
PubMed1994

Fetal congenital heart disease: diagnosis and management.

The most important papers address controversial aspects of fetal cardiology, for example, screening for congenital heart disease (CHD), the diagnosis and the accuracy of diagnosis, and the management of fetal tachycardias. There are some interesting additions to the literature although some conclusions require further evaluation.

Female↗
PubMed1981

[Fetal echocardiography: structural evaluation of the fetal heart and prenatal diagnosis of congenital heart disease (author's transl)].

Two-dimensional echocardiography has developed to the routine examination to diagnose congenital heart disease. In this study, we studied the value of two-dimensional echocardiography for evaluating the development of the fetal heart and for prenatal diagnosis of congenital heart disease. We studied 100 fetuses whose gestational ages were 16-40 weeks of pregnancy. The examination was performed with Toshiba Sonolayer-V model SSL-53M. The scanners were the 2.25 MHz linear and sector transducers. The images were recorded on videotapes. Changing the scanner direction according to the fetal position, we recorded the left ventricular long-axis view, short-axis view of great arteries and four-chamber view. In the four-chamber view, diameters of the tricuspid and mitral valve rings were measured. In the short-axis view of great arteries, the diameters of the aorta and pulmonary artery were measured. We identified the right and left ventricles by detecting both the great arteries and attaching sites of the atrioventricular valves. The identification of the aorta was made by detecting the arch and the identification of the pulmonary artery was made by depicting its two branches. Cardiac structures were identified on the fetus over five months of pregnancy. In many instances recordings were obtained in 7 months fetuses. The success rate to get clear recordings was 88% of all cases in the four-chamber view, 75% in the short-axis view of great arteries and 71% in the left ventricular long-axis view, respectively. The average ratio of the mitral to tricuspid valvular diameter and that of the pulmonary arterial to aortic diameter were both less than 1, suggesting the right ventricular dominance in fetal hearts. From these findings, it might be possible to diagnose prenatally certain congenital heart diseases such as atrioventricular valve atresia and semilunar valve atresia which require the critical evaluation and management in the neonatal periods.

Echocardiography↗
PubMed2001

Echocardiographic study of patients with congenital heart disease and infective endocarditis.

BACKGROUND: Infective endocarditis (IE) occurs with significant frequency in patients with congenital heart disease. The complications leading to increased morbidity and mortality may be detected by echocardiographic examination. This study was undertaken in order to identify echocardiographic findings that influence the outcome of patients with congenital heart disease and IE. METHODS: Twenty-five patients with an average age of 28 years were selected and divided into two groups according to evolution. Group I included patients who survived the infectious process, while Group II included patients who died during hospitalization or after release. RESULTS: Aortic valve disease was the most frequent anomaly. The clinical finding of most relevance for evolution during hospitalization was heart failure. Acute kidney failure and multiple organ failure from sepsis were the most common complications in patients who died. Echocardiograms established the diagnosis in all cases. Transesophageal studies revealed all periaortic abscesses. CONCLUSIONS: Echocardiography makes it possible to identify and evaluate complications associated with elevated morbidity and mortality in patients with congenital heart disease and IE.

Adolescent↗
PubMed1997

Life expectancy and social adaptation in individuals with Down syndrome with and without surgery for congenital heart disease.

Life expectancy and social adaptation in 373 children with Down syndrome with and without congenital heart disease (CHD) were assessed retrospectively. Survival at age 24 years was 92.2% for patients without CHD (n=200), and 74.6% for those with CHD (n=173). Survival for those who underwent operation for cardiovascular lesions (n=95) was 87.8%, and for those not operated on despite hemodynamically significant cardiovascular lesions (n=39), it was 41.4%. Cardiac functional capacity was better in the children without congenital heart disease and in the group operated on, where most patients also attained good social adaptation. We conclude that children with Down syndrome with congenital heart disease should undergo early cardiac evaluation and surgery if indicated.

Down Syndrome↗
PubMed2004

Implantable cardioverter defibrillators in adults with congenital heart disease: a single center experience.

Sudden death is the most frequent cause of late mortality in adults with congenital heart disease. This study reviews the experience of an Adult Congenital Heart Center with the use of implantable cardioverter defibrillators (ICDs). The charts of adults with congenital heart disease who had an ICD implantation were reviewed for diagnosis, residual lesions, reoperations, reason for implantation, complications, and recurrence of arrhythmias. Since 1995, 13 patients (mean age 43 years) had an ICD implantation for aborted sudden death (4), and spontaneous (6), or induced (3) ventricular tachycardia. Diagnosis were repaired (6) or palliated (1) tetralogy of Fallot, operated pulmonary stenosis (2), palliated complex pulmonary atresia (1), congenitally corrected transposition (1), operated ventricular (1), and atrial (1) septal defects. Significant residual lesions included severe pulmonary regurgitation (2), systemic ventricular dysfunction (2), and severe pulmonary hypertension (1). Five patients had a QRS > 180 ms, four had a QRS < 180 ms, four had a paced rhythm. Overall, four (31%) patients had no ventricular dysfunction, no residual lesion, and QRS < 180 ms. During a mean follow-up of 29 months, seven patients had recurrent ventricular tachycardia, three with normal ventricular function, no residual lesion, and QRS < 180 ms. ICD implantation is an important adjunct in the management of adults with congenital heart disease. As malignant arrhythmias occur even in patients with no residual lesion, no QRS prolongation and no ventricular dysfunction, the recognition of those who would benefit from an ICD remains a clinical challenge.

Adolescent↗
PubMed1998

Inoperable pulmonary vascular disease in infants with congenital heart disease.

BACKGROUND: Among 120 infants less than 12 months of age who had lung biopsy and autopsy, 20 were inoperable because of severe irreversible pulmonary vascular disease. METHODS: The infants were classified into three groups. Group 1 comprised 6 patients who showed complete obstruction of the small pulmonary arterial lumen and atrophy of the peripheral arterial media and who were considered to have absolute operative contraindications. Group 2 comprised 6 patients who had no pathologic findings of absolute operative contraindication and had an index of pulmonary vascular disease of more than 2.2. They were isolated as having advanced plexogenic pulmonary arteriopathy. Group 3 comprised 8 patients who had extremely thickened media of small pulmonary arteries, with abnormally thickened media extending into the small peripheral arteries characterized by extremely narrow lumina and medial thickness exceeding luminal diameter. RESULTS: Six of the 9 patients in whom operative repair was abandoned on the basis of preoperative or intraoperative lung biopsy are still alive. Of the 11 patients who underwent operation without biopsy, none survived. CONCLUSIONS: Preoperative or intraoperative lung biopsy and assessment of arteriopathy based on the above criteria are recommended in all patients in whom fatal pulmonary vascular disease is suspected.

Atrophy↗
PubMedDate not supplied

Issues related to prenatal diagnosis of congenital heart disease.

Enhanced technology and professional expertise are making the diagnosis of congenital heart disease possible with increasing frequency during the prenatal period. This article describes the process of prenatal diagnosis of congenital heart disease and the many personal issues families face when they receive news of this condition. Ethical and legal considerations of prenatal diagnosis are reviewed. Brief vignettes describe what families experience when they receive the diagnosis. The role of the nurse in educating parents, assisting them in sorting out options for treatment, and providing ongoing care and support is discussed, and implications for nurses in obstetric, neonatal, and pediatric settings are reviewed. Finally, the need for further research on this topic is explored.

Abortion, Eugenic↗
PubMed1990

Evaluation of surgical procedures for cyanotic congenital heart disease by using MR imaging.

ECG-gated MR imaging has been shown to be effective for the diagnosis of congenital heart disease. In this study, we assessed its role in the postoperative evaluation of surgical procedures in patients with complex congenital heart disease. MR images of 26 patients with Rastelli (five), Fontan (three), Senning (three), Damus (one), Jatene (eight), Waterston (four), and Potts (two) procedures were evaluated retrospectively. The accuracy of MR imaging was compared with that of angiography in 20 patients. The surgical anastomoses were identified in all patients. Patency, atresia, or hypoplasia of central pulmonary arteries and postoperative complications (focal stenoses of pulmonary arteries, thrombosed conduit, peri-conduit abscess) were shown. Narrowing of the right ventricular outflow tract and focal compression of the proximal pulmonary arteries were recognized as specific complications of the Jatene procedure. MR imaging appears to be effective in the postoperative evaluation of surgical procedures used for congenital heart disease. It should be considered as an alternative to repeated catheterization and angiography for the postoperative examination of children with complex congenital heart disease.

Adolescent↗