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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↗

PubMed1983

Laser irradiation of congenital heart disease: potential for palliation and correction of intracardiac and intravascular defects.

We examined the potential for laser irradiation of congenital heart defects, with the use of postmortem hearts and an argon ion laser with a flexible quartz fiber. Atrial septectomy was performed in five newborn hearts. Obstructive lesions were relieved by laser irradiation in valvular pulmonic and aortic stenosis, dysplastic pulmonary valve, pulmonary atresia, and coarctation of the aorta. To demonstrate the efficacy of in vivo cardiac laser surgery, atrial septectomy was also performed in an anesthetized dog model, under echocardiographic visualization, without change in heart rate or blood pressure. Our results demonstrate the feasibility of intracardiac and intravascular laser irradiation for palliation and repair of selected congenital heart diseases.

Animals↗

PubMed2003

Evaluation of the post-shunt status with electron beam computed tomography in cyanotic congenital heart disease.

The assessment of the accuracy of Electron Beam Computed Tomography (EBCT) for the follow-up of pulmonary vascular system after the shunt operation in cyanotic congenital heart diseases was purpose of the study. The study group consists of 16 consecutive patients with cyanotic congenital heart disease who had Blalock-Taussig (BT) shunt (n=7), bi- directional cavo-pulmonary shunt (BCPS, n=7) and unifocalization (n=2). EBT images were obtained on systolic phase under EKG gating and after intravenous administration of contrast agent. We evaluated the shunt patency, anatomy of intrapericardial pulmonary artery, parenchymal pulmonary vessels and background lung attenuation for the pulmonary blood flow, and the presence of systemic arterial and venous collaterals. Angiography (n=12) and echocardiography (n=16) were used as the gold standards. EBCT was consistent with angiogram in detecting the shunt patency and in depicting the anatomy of the intrapericardial pulmonary artery. Occlusion of the BT shunts was not detected in 2 patients by echocardiography. Diffuse or focally decreased pulmonary flow on EBCT in 8 patients was consistent with the pulmonary hemodynamics pattern revealed by cardiac catheterization. Uneven attenuation between lobes was related with multifocal supply of pulmonary flow or occlusion of lobar pulmonary arteries. Systemic collateral arteries were observed in 5 at the corresponding site of the decreased pulmonary flow. Systemic venous collaterals seen in all patients following BCPS were eventually draining to the inferior vena cava in 5 and to the left atrium in 2. EBCT provided accurate information of the pulmonary vascular system after shunt and has unique advantage over echocardiography in assessing patency of BT shunt or unifocalization tubes within the pleural space, the estimation of regional difference in pulmonary hemodynamics, and the detection of systemic collateral vessels. Therefore EBCT may provide useful information about the timing of definitive correction and the need for a second shunt or an interventional procedure prior to total repair.

Child↗

PubMed2002

Overproduction of platelet microparticles in cyanotic congenital heart disease with polycythemia.

OBJECTIVES: We sought to clarify the role of platelets in the pathogenesis of abnormal coagulation in patients with cyanotic congenital heart disease (CCHD) with polycythemia; we evaluated the production of platelet microparticles (MPs), platelet degranulation and aggregation response, as well as the correlations of these variables with polycythemia. BACKGROUND: A shortened life span and suppressed aggregability of platelets are well known in patients with CCHD. Although platelet MPs are overproduced and play an important role in the coagulation process in various hematologic and cardiovascular disorders, the production of MPs remains to be elucidated in CCHD. We studied 19 patients who had CCHD with polycythemia and 21 age-matched subjects with acyanotic congenital heart disease (ACHD). Flow cytometry, using monoclonal antibodies, showed the presence of MPs as particles positive for the surface antigen (glycoprotein IIb/IIIa) specific to platelets, and platelet alpha-degranulation was recognized as platelets positive for the surface antigen of P-selectin. Platelet aggregation was assessed as the response to adenosine diphosphate (ADP). Relationships between these indexes and hematocrit (Hct) values were also evaluated. RESULTS: Production of MPs correlated positively with Hct and markedly increased at Hct values above 60% in patients with CCHD. Surface P-selectin and the mean platelet volume in patients with CCHD were comparable with those in patients with ACHD. The platelet aggregation response to ADP significantly and negatively correlated with Hct. In two subjects who showed hemoptysis and underwent phlebotomy, MPs were reduced 6 h after the procedure. CONCLUSIONS: Platelet MPs are overproduced in patients who have CCHD with polycythemia, probably due to a high shear stress derived from blood hyperviscosity. Circulating incompetent platelets, which have already been activated, as well as MPs, might play an important role in the coagulation abnormalities identified in such patients.

Adolescent↗

PubMed1995

Radiofrequency ablation of intra-atrial reentrant tachycardia after surgical palliation of congenital heart disease.

BACKGROUND: Intra-atrial reentrant tachycardia (IART), also called atrial flutter, is a common and potentially lethal complication of surgical correction of congenital heart disease. Medical management of IART is often problematic, which prompts an investigation of the utility of radiofrequency (RF) ablation for management of these arrhythmias. METHODS AND RESULTS: Ten consecutive patients referred for treatment of recurrent IART after surgery for congenital heart disease were studied. Median age was 18.4 years, and median duration of arrhythmia was 6.4 years; a median of three antiarrhythmic drugs had been tried. Surgical procedures used were Fontan (6), Mustard/Senning (2), and biventricular repair (2). Intracardiac electrophysiological study demonstrated 30 distinct IART circuits, defined by activation sequence and cycle length. Mean IART cycle length was 323 +/- 114 ms. Cycle length was significantly longer in IART circuits that were successfully ablated compared with those that were not (381 versus 248 ms, P < .001). RF ablation was attempted in 22 of these circuits. Ablation sites were targeted to presumed exit points from zones of slow conduction by electrophysiological criteria. Sites chosen in this manner clustered in four distinct areas of the right atrium. Of 22 IART circuit ablations attempted, 17 (77%) resulted in acute termination of the tachycardia. In 8 of 10 patients in whom at least one IART circuit was successfully ablated, 4 are free of clinical tachycardia and 3 are improved over short-term follow-up. No complications were encountered. CONCLUSIONS: Multiple IART circuits may be present in patients after surgery for congenital heart defects. Activation sequences observed were diverse and different from those observed in atrial flutter in patients with normal anatomy. Interruption of IART circuits by RF ablation is feasible using mapping techniques aimed at identifying an exit point from a zone of slow conduction. Short-term follow-up suggests that RF ablation may be a useful adjunct in management of IART in these difficult patients.

Adolescent↗

PubMed1998

Detection of fetal congenital heart disease in a low-risk population.

Our purpose was to evaluate the efficacy of level two ultrasound screening for the detection of congenital heart defects (CHD) in a low-risk population by using three standardized cuts. Within a period of four years a total of 6727 pregnant women of a low-risk population undertook several ultrasound examinations on the basis of screening for fetal malformations. All ultrasound examinations were performed by three experienced doctors. At every single scan three standardized cuts (apical and lateral four-chamber view, crossing over of the great arteries) were obtained in order to detect congenital heart defects. Of 87 CHDs (1.33 per cent of the examined women) 39 (43.8 per cent) were diagnosed prenatally. The detection rate was 10/48 (20.8 per cent) in the presence of VSD, ASD2 or combined ASD2 + VSD, the detection rate was 29/39 (74.3 per cent) in the presence of other forms of congenital heart disease. None of the 38 missed cases in the first group but three of the ten missed CHDs in the second group had emergency neonatological problems. Aneuploidy and/or other malformations existed in 22/87 cases of CHD. The obstetrical management was changed in nearly all cases after the diagnosis of a CHD. Twenty-two women opted for termination of pregnancy because of additional fetal malformations or chromosomal defects. Five women were transferred prenatally to a tertiary centre for neonatal cardiac surgery. Ten deliveries were performed in the presence of a neonatologist. Good detection rates for CHD can be achieved in a low-risk population on the basis of level two ultrasound screening by using the above mentioned three cuts and thus, the perinatal mortality and morbidity can be improved.

Abnormalities, Multiple↗

PubMed2001

Illness-related concerns of mothers of children with congenital heart disease.

OBJECTIVE: This study examined concerns expressed by mothers of children with congenital heart disease (CHD). The relationships among mothers' concerns, medical severity, and mother's emotional state were examined at two points in time. METHOD: Thirty-eight mothers of children with CHD aged 3 to 16 completed semistructured interviews and rating scales during hospitalization and 2 to 4 weeks after discharge. Mothers rated their distress about illness-related concerns, as well as their own depressed mood and anxiety. Mothers and two cardiologists rated the medical severity of each child's disease. RESULTS: Mothers' concerns were reliably grouped into five categories: medical prognosis, quality of life, psychosocial functioning, effects on family, and financial issues. During hospitalization, mothers were most concerned about medical prognosis. Distress about most concerns decreased postdischarge, as did mother's anxiety and depressed mood. Mothers' perceptions of medical severity were associated with distress about psychosocial issues postdischarge. Mother's anxiety was not associated with number of concerns reported, or with distress about those concerns. Maternal depressed mood was associated with fewer illness-related concerns, but greater distress about those concerns. CONCLUSIONS: Illness-related concerns can be meaningfully categorized and are not necessarily a function of disease severity or mother's emotional state. An awareness of common concerns will improve clinical care by enabling practitioners to anticipate and address concerns in a proactive way. The results may inform the development of supportive mental health interventions for families of children with CHD.

Adaptation, Psychological↗

PubMed1994

Long-term follow-up of the postoperative patient with congenital heart disease. General concerns.

There are an estimated 400,000 adults with postoperative congenital heart disease (CHD) in the United States. The majority of these patients require long-term follow-up for management of persistent problems. These problems are related to electrophysiologic sequelae, myocardial changes, ventricular failure, prosthetic materials, and infective endocarditis. The goal for adults with postoperative CHD is to achieve not only long-term survival, but the best possible quality of life.

Adult↗

PubMed1979

The pediatrician and congenital heart disease--1979.

Technologic advances in the diagnosis and treatment of children with congenital heart disease have created new, expanded roles for the practicing pediatrician. Early identification of infants with serious lesions continues to be vital, and support of children and their families following surgery is central to the solution of emotional, employment, and childbearing problems these patients may eventually face.

Affective Symptoms↗

PubMed1986

Changing trends of congenital heart disease in adults: a catheterization laboratory perspective.

Advances in cardiac surgery now enable many children with congenital heart disease (CHD) to survive to adulthood. The influence of such advances on the frequency of various lesions among adult patients undergoing cardiac catheterization (CC) has not previously been addressed. This retrospective analysis of 329 adults with CHD undergoing CC at an adult and at a pediatric referral center demonstrates that when compared to 20 years ago, adults with CHD who now undergo CC are more likely to have complex cardiac disease, and more have had prior surgery. In addition, despite more aggressive surgical management, ventricular dysfunction and dysrhythmias are now commonly encountered residua of CHD in adults who require diagnostic CC. Premature coronary artery disease appears uncommon. These changing characteristics of adults with CHD require consideration in the planning of future health care for these patients.

Adult↗

PubMed2006

Prenatal screening for congenital heart disease with four-chamber and outflow-tract views: a multicenter study.

OBJECTIVE: Congenital heart diseases (CHD) are the most common congenital anomalies, and most cases occur in the low-risk population. Prenatal ultrasound screening based on visualization of the four-chamber view has had disappointing results in detecting these anomalies thus far. The aim of this study was to evaluate the diagnostic accuracy of ultrasound screening based on the combination of the four-chamber and outflow-tract views. METHODS: We conducted a multicenter prospective observational study in 15 obstetric units in the Piedmont Region, Italy. All operators received specific training. Data were recorded regarding visualization of the four-chamber view and the outflow tracts at each routine scan in pregnancies without any risk factor. When an anomaly was suspected, the patient was sent to the referral center. We obtained the follow-up data of the newborns until discharge from hospital and calculated the diagnostic accuracy of the test. RESULTS: 9074 ultrasound scans were performed on 7041 women and complete follow-up information was available for 6368 of them. Fifty-eight cases of CHD were observed at birth or postmortem (prevalence 9.1 per thousand); 38 of them were diagnosed in utero. The sensitivity of the test was 65.5%, the specificity 99.7%, the positive predictive value 70.4% and the negative predictive value 99.7%. The sensitivity of the four-chamber view alone was 60.3%. CONCLUSIONS: The sensitivity was significantly higher than that in a similar study performed in 1997 in the same setting. This improvement can be attributed in part to extension of the examination to the outflow-tract view, but also to technological developments and better training of the operators.

Echocardiography↗

PubMed2005

Physical performance and physical activity in grown-up congenital heart disease.

AIM: To review exercise performance and exercise habits in patients with congenital heart disease (CHD). BACKGROUND AND METHODS: Physical exercise and physical activity has shown beneficial effects on the physical, psychological and social level in adult patients with cardiovascular disease. Favourable effects have also been documented in children with CHD. Exercise testing is preferentially performed on a treadmill in children, with the measurement of gas exchange. RESULTS: An overview of the literature showed that formal exercise testing has frequently documented reduced or suboptimal values for aerobic exercise performance in children with left-to-right shunts (atrial septal defect, ventricular septal defect), valvular heart disease and obstructive anomalies (aortic stenosis, pulmonary stenosis, coarctation of the aorta). Subnormal values for exercise tolerance have also been observed in patients with successfully repaired cyanotic heart disease (tetraology of Fallot, transposition of the great arteries, Fontan operation). An important contributing factor to the impaired exercise performance is the hypoactive lifestyle, as often observed in patients with CHD. This frequently results from parental or environmental overprotection. CONCLUSION: These patients should be stimulated to be physically active, unless medical restriction is imposed. Fortunately, this represents only a small fraction of the total number of congenital heart defects for which sports participation is allowed.

Adult↗

PubMed1989

[Familial malformation recurrence in congenital heart disease].

The intensity and degree of familial association in a series of congenital heart diseases has provided 204 cases of familial malformation recurrence. There was total concordance for 27% of first degree relatives, 18.5% of those of second degree, and 9% of third degree; partial concordance was present in 15, 18 and 41%; system concordance in 27, 44, and 43%; total discordance in 31, 18.5, and 5%. Anencephaly, hydrocephaly, cleft lip-palate, pyloric stenosis, and cryptorchidism were repeatedly present with a highly significant incidence.

Anencephaly↗

PubMed1982

Gastro-cutaneous syndrome: peptic ulcer/hiatal hernia, multiple lentigines/café-au-lait spots, hypertelorism, and myopia.

We report the familial occurrence in a French Canadian family of peptic ulcer/hiatal hernia, multiple lentigines/café-au-lait spots, apparent hypertelorism, and myopia caused by a pleiotropic autosomal dominant gene with high penetrance and variable expressivity. Other probable but rarer components of the syndrome include ischemic heart disease, congenital heart disease, and maturity onset diabetes. Symptoms of peptic ulcer/hiatal hernia usually started in the second or third decade and were associated, as least in some examined individuals, with relatively increased acid secretion and abnormal dermatoglyphics.

Adolescent↗

PubMed1985

Clinical and echocardiographic observations in pulmonary valve endocarditis.

Clinical and echocardiographic data from 12 patients with pulmonary valve endocarditis are described. Seven patients had isolated pulmonary endocarditis and in 5 patients other valves were infected (aortic, tricuspid, mitral or all 3). Two patients were heroin addicts and 4 had underlying heart disease (congenital heart disease in 3 and aortic regurgitation in 1 patient). The organisms involved were alpha streptococci in 3 patients (all with underlying heart disease), Staphylococcus aureus in 4, Streptococcus D bovis in 1 patient and Candida guillermondii in 1. M-mode and 2-dimensional echocardiography was performed in 10 patients and revealed vegetations in 8. Pulsed Doppler echocardiography was performed in 6 patients and revealed pulmonary regurgitation in all 6. Seven patients had pulmonary emboli. Four patients underwent surgery. Four patients died, including 1 after cardiac surgery. Five patients, including the patient infected with Candida guillermondii, recovered with antibiotic treatment.

Adolescent↗

PubMed2007

Interventional therapies for heart failure in the elderly.

The aging of a population replete with risk factors for heart failure(HF) (coronary heart disease, diabetes, and hypertension) coupled with a declining age-adjusted mortality rate for coronary artery and hypertensive heart diseases has created, and will continue to create, a literal explosion in the prevalence of HF in the United States. Despite advances in maximal medical therapy, however, most patients who have symptomatic HF can expect functional impairment, interludes of worsening symptomatology, and a shortened lifespan. This article updates the use of interventional therapies for the treatment of elderly patients who have HF caused by coronary artery disease, valvular heart disease, congenital heart disease, myocardial disease, and renal vascular disease.

Aged↗

PubMed1976

Electrocardiographic and hemodynamic correlations in patients with idiopathic hypertrophic subaortic stenosis.

The ECG and Frank VCG were compared to the hemodynamic findings in 33 patients with idiopathic hypertrophic subaortic stenosis in whom cardiac catheterization had excluded concomitant valvular heart disease, congenital heart disease, or occlusive coronary artery disease. The patients were divided into two groups according to the absence (Group I) or presence (Group II) of left ventricular hypertrophy on the ECG and/or VCG. The 11 patients in Group I were found to have neither mitral insufficiency nor a resting left intraventricular gradient, and only six patients in whom mitral valve movement was visualized demonstrated systolic anterior movement of the anterior leaflet. The papillary muscles and left ventricular wall were either normal or only mildly hypertrophied in 10 of 11 Group I patients. Group II (22 patients) demonstrated either a resting left intraventricular gradient and/or mitral insufficiency in 18 patients. Twenty-one of the 22 patients showed systolic anterior movement of the anterior leaflet of the mitral valve on a cineangiogram and the papillary muscles and left ventricular wall were moderately to severely hypertrophied in 18 patients. These data suggest that specific hemodynamic and anatomic characteristics of hypertrophic subaortic stenosis may be predicted with reasonable accuracy from the ECG and VCG.

Adult↗

PubMed1990

Right aortic arch with isolation of the left subclavian artery: case report and review of the literature.

Of the right aortic arch anomalies, a right arch with isolation of the left subclavian artery is the least common. Herein we describe a 52-year-old woman in whom this anomaly was discovered during cerebral angiography for evaluation of a giant symptomatic intracavernous carotid aneurysm. Isolation of the left subclavian artery may be suggested in a patient with a right arch in whom the blood pressure or pulse in the left upper extremity is diminished. Although the isolated left subclavian artery produces the hemodynamic alterations of a subclavian steal, review of the 39 cases reported in the literature revealed only 5 patients with symptoms suggestive of vertebrobasilar insufficiency and 5 patients with weakness of the left upper extremity. Although the patient we describe had no known heart disease, congenital heart disease was present in 23 of the 39 reported cases (59%), tetralogy of Fallot occurring most frequently.

Aneurysm↗