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PubMed2004

Patterns of congenital heart disease in unoperated adults: a 20-year experience in a developing country.

BACKGROUND: The number of patients with congenital cardiac disease reaching adulthood is increasing steadily. Many adults with such disease face both medical and surgical difficulties. HYPOTHESIS: This retrospective study was undertaken to assess the frequency and outcome of congenital heart disease (CHD) in unoperated adults. METHODS: The charts of all patients with unoperated CHD, who were admitted to a tertiary care center in Beirut, Lebanon, between 1980 and 2000 were reviewed. Of these, 206 patients (52% men, age at admittance 18-71 years [32.8 +/- 13.3 years]) with a diagnosis of CHD were evaluated. Atrial septal defect (ASD) was the most common cardiac malformation with a relative frequency of 53%, followed by ventricular septal defects (11%), tetralogy of Fallot (11%), aortic anomalies (7%), pulmonary stenosis (6%), and Ebstein anomaly (4%). Most patients were symptomatic upon presentation, with dyspnea on exertion being the most common presenting symptom. Twenty-seven patients (13%) had cyanotic CHD. Of 179 acyanotic patients, 113 (63%), and 17 of 27 cyanotic patients (63%) underwent surgical intervention. In-hospital surgical complications for the acyanotic group included cerebrovascular accident (2%) and heart block (1%). Total surgical mortality was 4 of 130 (3%). One patient with tetralogy of Fallot presented with endocarditis and died. CONCLUSION: Atrial septal defect is the most common defect reported in our experience; however, it occurs more frequently than that reported in the literature. Although most patients were symptomatic on presentation, their functional status was stable. Accordingly, their hospital course, whether managed medically or surgically, held a relatively low complication rate. This could be attributed to the uncomplicated nature of pathologies in our series. The surgical mortality and in-hospital complications were slighter higher than those reported for similar lesions if repaired during childhood. This study reflects the relative frequency of various cardiac malformations in selected patients with "grown up" congenital heart disease (GUCH) and their natural survival pattern.

Adolescent↗
PubMed2002

Knowledge, compliance and practice of antibiotic endocarditis prophylaxis of patients with congenital heart disease.

The aim of this study was to determine the knowledge, compliance, and practice of antibiotic endocarditis prophylaxis (AEP) for patients with congenital heart disease (CHD) during various diagnostic or therapeutic procedures. Patients (296) and their parents were interrogated during a visit with an 18-question survey pertaining to the practice of AEP. Most patients (91.6%) had acyanotic congenital heart disease, usually preoperative (45.3%), or had high risk of infective endocarditis due to previous endocarditis (2.0%), cyanosis (8.4%), or prosthetic valves (5.4%). Potential sources for failure of AEP were (1) no existence of a wallet card (6.8%), no communication between patient and doctor concerning the potential risk of endocarditis during a visit (13.2%), or no sufficient anamnestic inquiry by the physician before procedures were performed (43.2%). The patients underestimated (4.4%) or had no knowledge of the individual risks of endocarditis (37.5%) or underestimated indication for AEP (11.1%). AEP was often not performed at all (60.5%). Bad dental status with no previous dental therapy (35.5%) and a high rate of caries (17.2%) or gingivitis (7.4%) even in young children was frequent. AEP for patients with CHD has not been sufficiently used because of the patient's, and the physician's failure to take the necessary steps, the cardiac diagnosis, or the dental health status. Alternative strategies in prophylaxis for AEP are discussed here.

Adolescent↗
PubMed1992

Digital subtraction angiography in the preoperative diagnosis of congenital heart disease.

From December of 1986 through February of 1990, digital subtraction angiography was performed on 156 patients, and specific diagnoses of congenital heart disease were made on the basis of information gathered by this imaging technique, supplemented by the physical examination. No other imaging techniques were used, for this was a prospective study designed to determine the accuracy of digital subtraction angiography alone in the diagnosis of congenital heart disease. When 80 of these 156 patients underwent subsequent operation, 75 were shown to have been diagnosed correctly, for an accuracy rate of 93.75%. Two patients died, for a mortality rate of 2.5%. The diagnostic value of digital subtraction angiography is discussed, as well as the compensative management of its artifacts.

Journal Article↗
PubMed2006

[Prenatal diagnosis of congenital heart disease and its consequences].

Echocardiography allows the prenatal diagnosis of most congenital cardiac malformations. Although the indications for this specialized ultrasound technique are multiple, suspicion of congenital heart disease at the second trimester ultrasonographic screening has become the most frequent indication. Specially trained pediatric cardiologists can confirm the diagnosis or rule it out. If the diagnosis is confirmed, then a fetal karyotype should be proposed in most cases and thorough examination of extracardiac organs, should be performed. If the prognosis is poor, French law authorizes termination of pregnancy. If the pregnancy goes on, a multidisciplinary team should organize the perinatal care according to expected neonatal difficulties.

Female↗
PubMed1983

Mesangiolysis in the glomerular lesion of cyanotic congenital heart disease.

Mesangiolysis associated with glomerulomegaly was observed in an autopsy case of an 11-year-old boy with cyanotic congenital heart disease, i.e. complete transposition of the great vessels with large VSD. Microscopical examination of the kidney revealed marked enlargement of glomeruli with mesangiolysis mainly found in the midzonal cortex involving 16 to 20% of all glomeruli. The glomerular basement membrane in the mesangiolytic lesion showed splitting, between which red blood cells were occasionally encountered. By reconstruction study with serial sectioning, the mesangiolysis was observed to be obviously focal and segmental. Immunofluorescent study revealed no positive results for IgG, IgA, IgM, C3, and fibrinogen/fibrin. By electron microscopic study on the post formalin fixed specimens, no remarkable dense deposits were found in any structural components of the glomeruli. It was assumed that the mesangiolysis might have been provoked by some circulatory disturbances, which superimposed on the preexisting glomerular lesion of cyanotic congenital heart disease in the terminal stage of the clinical course.

Child↗
PubMed2006

Blood viscosity and its relationship to iron deficiency, symptoms, and exercise capacity in adults with cyanotic congenital heart disease.

OBJECTIVES: This study sought to determine the relationship between blood viscosity and iron deficiency and their impact on symptoms and exercise function in adults with cyanotic congenital heart disease. BACKGROUND: Iron deficiency is believed to raise whole blood viscosity in cyanotic congenital heart disease, although available data are inconsistent. METHODS: Thirty-nine cyanotic adults were prospectively assessed for iron deficiency (transferrin saturation < or =5%), hyperviscosity symptoms, and exercise capacity. Same-day measurement of whole blood viscosity and hematocrit (Hct) adjusted viscosity (cells resuspended in autologous plasma to Hct of 45%) was performed at shear rates ranging from 0.277 s(-1) to 128.5 s(-1). RESULTS: Viscosity did not differ between patients with iron deficiency (n = 14) and those without (n = 25). Whole blood viscosity correlated with Hct (r = 0.63, p < 0.001 at low shear and r = 0.84, p < 0.001 at high shear) but not with red blood cell size or iron indices. Hyperviscosity symptoms were independent of iron indices but directly correlated with increased Hct-adjusted viscosity (r = 0.41, p = 0.01). Exercise capacity did not differ in iron-deficient patients. However, peak oxygen consumption was higher in those with Hct > or = 65% (12.6 +/- 3.4 ml/kg/m2 vs. 9.8 +/- 2.6 ml/kg/m2, mean +/- SD, p = 0.036) despite higher whole blood viscosity in these same individuals (p < 0.01 for all shear rates). CONCLUSIONS: Iron deficiency is common in cyanotic adults but does not alter viscosity. Hyperviscosity symptoms are associated with a higher Hct-adjusted viscosity independent of cell size or iron stores. Higher Hct is associated with better exercise capacity. Further work to understand the origin of hyperviscosity symptoms is warranted.

Adult↗
PubMed2004

The coronary circulation in cyanotic congenital heart disease.

BACKGROUND: Dilated coronary arteries, coronary blood flow, and the paucity of coronary atherosclerosis have not been studied in cyanotic congenital heart disease. METHODS: Coronary arteriograms were interpreted in 59 cyanotic adults, and dilated coronaries were examined histologically in 6. Coronary blood flow was determined with N-13 ammonia positron emission tomography in 14 Eisenmenger syndrome patients and in 10 controls. Total non-fasting cholesterols were retrieved in 279 patients who were divided into: Group A--143 cyanotic unoperated, Group B--47 acyanotic after operation, Group C--41 acyanotic unoperated, Group D--48 acyanotic before and after operation. Total cholesterol was <160 mg/ml in 58% of Group A and 51% of Group B. Low- (LDL) and high-density cholesterol (HDL) and triglycerides were determined in 57/82 hypocholesterolemic patients. Platelet counts were determined in 105 patients. Platelet production, megakaryocyte production, platelet destruction, and platelet activation were studied. RESULTS: Angiography--88% of extramural coronary arteries were mildly or moderately dilated to ectatic and tortuous. Loss of medial smooth muscle, increased medial collagen, and duplication of internal elastic lamina were identified histologically. Basal coronary flow was increased, but hyperemic flow following IV dipyridamole was comparable in patients and controls. Atherosclerosis was not detected in either the arteriograms or the necropsy specimens. Thrombocytopenic resulted from reduced platelet production. CONCLUSIONS: Coronary arteries in cyanotic congenital heart disease dilate in response to endothelial vasodilators coupled with mural attenuation caused by medial abnormalities. Basal flow was increased, but flow reserve was normal. Coronary arteries were atheroma-free.

Adolescent↗
PubMed1994

Infective endocarditis. Implications for care of the adult with congenital heart disease.

Infective endocarditis (IE) remains a severe complication for the adult with congenital heart disease (CHD). The last four decades have witnessed a radical transformation in the spectrum of IE, including the advent of cardiac surgery and the postantibiotic era. Because of the devastation caused by this disease, prevention is the hallmark. A clear understanding of the pathogenesis, clinical features, and management of IE is essential for any practitioner caring for adults with CHD.

Adult↗
PubMed1996

Homografts in congenital heart disease: current applications and future directions.

The first clinical use of homograft tissue in cardiovascular surgery was in 1948, when Gross used cadaveric arterial grafts to construct systemic to pulmonary artery shunts in patients with tetralogy of Fallot, and to repair coarctation of the aorta. Eighteen years later, a valved homograft was used for the first time in the treatment of congenital heart disease for reconstruction of the right ventricular outflow tract in a child with pulmonary atresia. Since these pioneering advances, valve and vascular homografts have become central to the management of congenital anomalies of the heart and great vessels. The primary use for homografts in congenital heart surgery today is establishment of a valved connection between the right ventricle and pulmonary arteries in children with tetralogy of Fallot with pulmonary atresia or other complicating factors, truncus arteriosus, transposition complexes, and double-outlet right ventricle, and in patients undergoing the Ross procedure. Homograft reconstruction of the left ventricular outflow tract has also been performed for many years in children with aortic insufficiency or recurrent aortic stenosis, but early homograft degeneration in young children has been a significant problem. Many surgeons are turning away from homografts in the systemic circulation in favor of the pulmonary autograft. Homograft is also widely used as a vascular patch material. In the present report, we discuss the various uses of homografts in congenital heart surgery, the benefits and drawbacks of homografts in young patients, and some of the future possibilities for homograft development and application in patients with congenital heart disease.

Adolescent↗
PubMed2003

Survey of prophylaxis and management of infective endocarditis in patients with congenital heart disease: Japanese nationwide survey.

Guidelines for the prevention and management of infective endocarditis (IE) in children with congenital heart disease (CHD) have not been established, so the aim of this study was to clarify the incidence, practical prevention and management of IE in patients with CHD in Japan through a nationwide survey. A written questionnaire was sent to members of the Japanese Society of Pediatric Cardiology and Cardiac Surgery and information was obtained from 236 cardiologists in 228 institutions. Four hundred and eight patients with IE were hospitalized during 1997 to 2001 (1/173 admissions with CHD including those hospitalized for cardiac catheterization or surgery). Prevention of IE for CHD was undertaken by 92% of cardiologists, usually oral penicillins (73%) and less frequently cephems (18%) were prescribed. The Duke criteria were used as clinical criteria by 38%. Blood culture was performed once only by 40%. Penicillins and aminoglycosides (38%) were frequently administered for management of culture-negative IE. There were variations in the dose and duration of antibiotics for prevention and management of IE. It appears that the prevalence of IE in CHD is rising and the nationwide survey revealed more variations in practical prevention and management of IE in patients with CHD than expected. The results should be helpful in making future guidelines for management of IE in CHD.

Administration, Oral↗
PubMed1986

[Present status and future view of cardiac surgery in Japan].

Analysis of present status: The institutes for cardiac surgery, amounting to 300 are spread throughout Japan. Consequently, the number of operation for the acquired heart disease, congenital heart disease and thoracic aortic aneurysm, has been counted 8088, 7837, and 839 respectively and in total, 16774 cases were operated in 1984. The increase in number of surgery for new born baby and infant recently proves the remarkable advance of cardiac surgery in Japan. The gradual increase in number of surgery for the aged reflects the Japanese social conditions today. The increase in number of aorto coronary bypass surgery should be paid attention, from the view of the disease structure in Japan. The problems, existing under today's condition are as follows; the establishment of the other department concerned with that of cardiac surgery, training condition to become specialists, practical use of medical resources and economical efficacy of medical care. Future view: It should be noted that the severe patients exist in Japan as well as in other countries and their lives cannot be saved without the heart or the heart-lung transplantation. We should immediately consider the organ transplantation as one of the medical treatments for the above-mentioned patients out of love for mankind. Heart transplantation is very much different from the general cardiac surgery in technical, ethical aspects and so on. Therefore, as the procedure for the success to establish heart transplantation as one of the medical treatments, careful consideration is indispensable in the nationwide.(ABSTRACT TRUNCATED AT 250 WORDS)

Cardiac Surgical Procedures↗
PubMed2001

Complete transposition of the great arteries: patterns of congenital heart disease in familial precurrence.

BACKGROUND: Transposition of the great arteries (TGA) is considered to be associated only rarely with genetic syndromes and to have a low risk of precurrence among relatives of affected patients. Because most family studies have involved a relatively small number of patients and evaluated all types of TGA as a single group, we performed a large, prospective study investigating the precurrence of congenital heart disease in families of children with complete, nonsyndromic TGA. METHODS AND RESULTS: From January 1997 through December 2000, 370 patients with nonsyndromic, complete TGA were consecutively evaluated and enrolled in the study. The occurrence of cardiac and noncardiac anomalies among relatives of the probands was investigated. Relatives with congenital heart disease were found in 37 of 370 families (10%), including 5 of 37 families (13.5%) with more than one affected relative. TGA itself was the most common precurrent malformation: complete TGA occurred in 6 families and congenitally corrected TGA occurred in 5 families. Precurrence risks for congenital heart disease were calculated at 1.8% (8 of 436) for siblings, 0.5% (4 of 740) for parents, 0.5% (16 of 3261) for first cousins, 0.2% (4 of 2101) for uncles/aunts, and 0.06% (1 of 1480) for grandparents. CONCLUSIONS: The present study shows that TGA is not always sporadic in families. Precurrence of concordant cardiac defects within affected family members supports monogenic or oligogenic inheritance of TGA in certain kindreds. Moreover, the occurrence of complete TGA and congenitally corrected TGA among first-degree relatives in several different families strongly suggests an underlying pathogenetic link between these 2 malformations that has been previously unrecognized.

Adolescent↗
PubMed1985

The relationship of congenital heart disease and respiratory infection mortality in patients with Down's syndrome.

Is respiratory infection mortality in Down's syndrome (DS) individuals due mainly to their congenital heart disease (CHD) or to other factors which subject most mentally retarded persons to risk? Detailed clinical and autopsy records of 137 institutionalized DS patients and 480 non-DS controls over 31 years yielded 42 DS subjects and 13 non-DS controls with congenital heart disease. These were compared to 20 DS patients and 20 controls without CHD. The DS and non-DS patients were matched for age, sex and IQ. DS patients had more CHD; controls had more pulmonary oedema. In neither group was there association between heart disease and death from respiratory infection. Nor did pulmonary oedema contribute importantly to such deaths. Such mortality, however, was associated with young age, short institutionalization and bedridden status. We conclude that respiratory infection death in DS individuals is due not primarily to heart disease but to factors which lead to mortality in the general population of retarded people.

Adolescent↗
PubMed1999

Pediatric cardiac extracorporeal membrane oxygenation in congenital heart disease: the cardiologist's view.

There is no doubt that extracorporeal membrane oxygenation (ECMO) as a powerful therapeutic modality in critically ill newborn infants and older children with congenital heart disease has implications for the pediatric cardiologist. His responsibilities as consultant in the intensive care unit include screening for unsuspected cyanotic heart disease in neonatal candidates referred for ECMO and appraisal of surgical repair in postcardiotomy patients as well as assessment of postoperative hemodynamics and detection of complications during perfusion. A close cooperation between intensive care specialists and other appropriate specialists (pediatric cardiologists, cardiac surgeons, and anesthesiologists) is required for the process of decision making prior to initiation of postoperative ECMO in the individual patient with congenital heart disease. Long-term survival, morbidity, cerebrovascular complications, and neurodevelopmental sequelae of these near miss children remain a critical issue. Furthermore, there is a strong need for professional psychosocial support of affected parents, both in the hospital and after discharge.

Brain Diseases↗
PubMed2002

Bronchial compression by posteriorly displaced ascending aorta in patients with congenital heart disease.

BACKGROUND: We encountered several patients with posteriorly displaced ascending aorta and bronchial compression associated with congenital heart disease. We describe the helical computed tomography (CT) findings and explore the mechanism of airway compression. METHODS: We retrospectively reviewed the clinical data and CT findings of 8 patients with posterior displacement of the ascending aorta. The bronchial stenosis was quantified on reformatted images perpendicular to the main-stem bronchi. On an axial image at the level of main bronchi, we measured depth of retrosternal space, interaortic distance, and aorto-spinal distance. To compare with control, we measured the same variables in 10 control patients. RESULTS: In 7 patients, the main bronchus on the side of the aortic arch was squeezed between the ascending and descending aorta and showed slit-like stenosis. The right pulmonary artery was elongated around the ascending aorta in 5 patients and showed slit-like stenosis in 3. Patients with posterior displacement had significantly larger retrosternal space, smaller interaortic distance, and smaller aorto-spinal distance than did the control group. Aortopexy was undertaken in 3 patients. Follow-up computed tomograms of 2 patients showed improvement. CONCLUSIONS: The posteriorly displaced ascending aorta may compress the main bronchus on the side of the aortic arch and right pulmonary artery against the descending aorta or spine. Even if the bronchial compression is mild with tolerable airway symptoms, these patients must be closely observed. When airway symptoms are severe, aortopexy should be considered.

Adolescent↗
PubMed1984

Digital subtraction angiography in infants and children with congenital heart disease.

Digital subtraction angiography was used as the sole imaging technique in 95 infants and children aged 13 hours to 16 years undergoing cardiac catheterisation for the investigation of congenital heart disease. Injections of diluted contrast medium were made selectively at central sites, and the images were obtained using continuous image intensification fluoroscopy at either 32.25 nC/kg/s (125 microR /s) or 129 nC/kg/s (500 microR /s). In all cases images adequate for diagnosis and management were obtained with appreciably less contrast medium and a lower radiation dose than in a comparable group of patients using conventional biplane cineangiography. Thus digital subtraction angiography is a viable alternative to biplane cineangiography for children with congenital heart disease.

Adolescent↗