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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↗
PubMed2000

Limitation of transthoracic echocardiography in the diagnosis of congenital heart diseases.

Nowadays, it has been widely accepted that echocardiography is the most efficient, non invasive diagnostic tool to diagnose congenital heart diseases. However, cardiac catheterization remains the gold standard to diagnose and obtain hemodynamic data prior to cardiovascular surgery. In order to find out the accuracy of transthoracic echocardiography in relation to the anatomical diagnosis of congenital heart diseases, 175 consecutive patients who underwent diagnostic cardiac catheterization during January 1999 to December 1999 were reviewed. All of them had complete echocardiographic studies prior to the procedure. The male to female ratio was 1.06:1. The median age at the time of echocardiography was 3.36 (0.01-28.8) years old. The indications of the cardiac catheterization were to demonstrate cardiovascular anatomy 64 per cent, to obtain pulmonary artery pressure and pulmonary vascular resistance 13.7 per cent, and to get both information 22.3 per cent. Tetralogy of Fallot (23.4%) was the most frequent cardiac malformation, followed by complex congenital heart diseases (22.3%), simple left to right shunt (12%), pulmonary atresia with ventricular septal defect (8.6%), tricuspid atresia (5.7%), simple d-transposition of great arteries (4%), etc. From cardiac catheterization; 49 cases (28%) revealed additional data which were surgically important, 3 cases (1.7%) resulted in different diagnoses, and 3 cases (1.7%) revealed additional information which was not surgically important. Inadequate imaging technique (36 cases, 65.5%) and limitation of technique (19 cases, 34.5%) were the reasons for missing anatomical findings of transthoracic echocardiography. Age at the time of echocardiography was not a significant factor affecting the accuracy. Persistent left superior vena cava, multiple aorto-pulmonary collateral arteries, pulmonary artery anatomy, and coronary artery anatomy were the most frequent cardiac lesions misdiagnosed by transthoracic echocardiography that were somewhat surgically important. The incorrect echocardiographic diagnoses were aorto-pulmonary window, patent ductus arteriosus, and vascular ring. Thorough and extensive echocardiographic scanning coupled with cooperative or adequately sedated patients by an experienced operator using an efficient echocardiographic machine might improve the accuracy of transthoracic echocardiography in the diagnosis of congenital heart diseases.

Adolescent↗
PubMed1992

Surgical management of congenital heart disease in the 1990s.

OBJECTIVE: To provide a broad overview of recent trends in surgical management of congenital heart disease. DESIGN: Review of recent literature. SETTING: Major centers for surgical management of congenital heart disease. SELECTION PROCEDURES: Analysis of important recent clinical publications. INTERVENTIONS: None. RESULTS AND CONCLUSIONS: Definitive, corrective surgery was previously reserved for children beyond infancy. Recognition of the complications associated with palliative operations as well as the realization that neonates and infants did not fare worse than older children during open heart surgery provided the impetus for primary corrective surgery at an earlier age. Advances in our understanding of perioperative care, improved diagnostic capability, and increasing experience with corrective surgery in association with several different congenital heart lesions has resulted in an established therapeutic approach to surgical management of congenital heart disease in neonates and infants.

General Surgery↗
PubMed1994

Transesophageal echocardiographic detection of atrial thrombi in patients with nonfibrillation atrial tachyarrhythmias and congenital heart disease.

OBJECTIVES: We hypothesized an association between atrial thrombi and nonfibrillation atrial tachyarrhythmias in patients with congenital heart disease. BACKGROUND: We observed a fatal thromboembolus after direct current cardioversion in an adolescent with atrial flutter and repaired tetralogy of Fallot. METHODS: Using transesophageal echocardiography, we prospectively studied 19 consecutive patients with congenital heart disease with nonfibrillation atrial tachyarrhythmia (atrial flutter in 18, primary atrial tachycardia in 1) undergoing electrophysiologic procedures (median age 19.6 years, range 7.0 to 53.8; 11 male, 8 female). Transthoracic echocardiograms were available for 17 patients. RESULTS: All transesophageal examinations were performed without incident. No atrial thrombi were detected in 11 patients who subsequently had uncomplicated direct current cardioversion. Eight solitary atrial thrombi were detected (incidence 42%). Six thrombi were located in the right atrium (Fontan repair in four patients, Ebstein's malformation repair in two), and two were noted in the left atrium (congenital hypertrophic cardiomyopathy and atrial septal defect repair in one patient each). Transthoracic echocardiograms were available in seven of eight patients with thrombus detected by transesophageal echocardiography, with only one study conclusive for an atrial thrombus. Cardioversion was deferred in six of eight patients with thrombus, and anticoagulation therapy was initiated. Uncomplicated electrophysiologic procedures were conducted in two patients at the time of detection of right atrial thrombus (atrioventricular node ablation in one patient, direct current cardioversion in the other). CONCLUSIONS: Prothrombin conditions exist in patients with congenital heart disease with nonfibrillation atrial tachyarrhythmias, as indicated by a significant incidence of transesophageally detected atrial thrombi. The need for prophylactic anticoagulation and the safety of pharmacologic or direct current cardioversion are issues that remain unresolved.

Adolescent↗
PubMed1986

Occlusion of intra-acinar pulmonary arteries in pulmonary hypertensive congenital heart disease.

Lung biopsy specimens from 10 consecutive cases of congenital heart disease and pulmonary hypertension (2 months-6.5 years) were examined by light and electron microscopy. By light microscopy, 8 cases showed a reduction in number of patent alveolar wall arteries associated with the structural features of early pulmonary vascular disease-extension of muscle, a significant increase in pulmonary arterial medial thickness (P less than 0.001) and little if any intimal proliferation (Heath & Edwards Grade I or II). In one additional child with Grade IV disease, arterial number could not be determined accurately. For all 10 cases, all blocks of tissue examined by light and electronmicroscopy showed absence of any lumen in structures identified as alveolar wall arteries by their position, shape and the presence of an external (or single) elastic lamina. In 3 additional similar cases, endothelial cells of narrowed and occluded arteries were marked by labelling with the lectin Ulex europaeus. Occluded arteries were identified consisting of mature smooth muscle cells and remnants of degenerating endothelial cells. Occlusion of alveolar wall arteries appears to be an early event in the development of pulmonary vascular disease, is associated with differentiation of smooth muscle cells in normally thin walled precapillary arteries and precedes significant obliterative intimal changes in more proximal vessels.

Arterial Occlusive Diseases↗
PubMed2005

Nurse specialists in adult congenital heart disease: the current status in Europe.

AIM: Recommendations for the management of adults with congenital heart disease indicate that specialist referral centres should employ nurse specialists who are trained and educated in the care for these patients. We surveyed the involvement, education and activities of nurse specialists in the care for adults with congenital cardiac anomalies in Europe. METHODS: The Euro Heart Survey on Adult Congenital Heart Disease has previously showed that 20 out of 48 specialist centres (42%) have nurse specialists affiliated with their programme. Fifteen of these 20 centres (75%) validly completed a web-based survey tool. RESULTS: Specialist centres had a median number of 2 nurse specialists on staff, corresponding with 1 full-time equivalent. In most centres, the nurse specialists were also affiliated with other cardiac care programmes, in addition to congenital heart disease. The involvement of nurse specialists was not related to the caseload of inpatients and outpatient visits. Physical examination was the most prevalent activity undertaken by nurse specialists (93.3%), followed by telephone accessibility (86.7%), patient education (86.7%), co-ordination of care (73.3%), and follow-up after discharge (73.3%). Patient education covered mainly prevention and prophylaxis of endocarditis (100%), cardiovascular risk factors (92.3%), sport activities (92.3%), the type and characteristics of the heart defect (92.3%), the definition and aetiology of endocarditis (84.6%), cardiac risk in case of pregnancy (84.6%), and heredity (84.6%). Two third of the nurse specialists were involved in research. CONCLUSION: This survey revealed gaps in the provision of care for these patients in Europe and demonstrated that there is room for improvement in order to provide adequate chronic disease management. The results of this study can be used by individual hospitals for benchmarking.

Adult↗
PubMed2004

Congenital heart disease in the adult: 2004.

The number of newborn infants with congenital heart disease is relatively stable.However, because of marked advances in correction and palliation of these conditions,the great majority will survive into adult life, significantly increasing the number of such patients in our population. Most patients with congenital heart disease, treated or not,will require routine, continued medical observation and, therefore, many radiologists will become involved in their care. Not uncommonly, pertinent clinical information is not provided to the radiologist, particularly when the patient presents to an Emergency Room because of intercurrent disease. In order to interpret the chest film or CT examination and provide a pertinent report, the radiologist must be conversant with the patterns of the more common congenital cardiac anomalies as well as their post-operative appearances and potential complications.

Adult↗
PubMed2002

[Cryopreserved viable pedicled aortic homografts for complex congenital heart diseases: report of 2 cases].

OBJECTIVE: To report the authors' experience in treating complex congenital heart diseases with cryopreserved viable pedicled aortic homografts (CVAHs). METHODS: CVAHs were obtained within 3 h after death of donors younger than 35 years old due to non-cardiovascular and non-infectious diseases. After routine treatment, the homografts were cryopreserved in liquid nitrogen and thawed in 0.9% sodium chloride solution at 37 to 42 degrees celsius before use. A CVAH was used as a valved conduit to connect the pulmonary artery and right ventricle in one case of double-outlet right ventricle, pulmonary stenosis and coronary artery deformation. In another case of severe tetralogy of Fallot, a CVAH was used as a valved patch to enlarge the right ventricle outlet. RESULTS: The homografts were preserved satisfactorily. The surgical results of the 2 cases with congenital heart diseases were satisfactory in perioperative and follow-up periods. CONCLUSION: CVAH is a good substitute to rebuild right ventricle-pulmonary artery connection in treatment of complex congenital heart diseases.

Aorta↗
PubMed1996

Sudden cardiac death in the adult with congenital heart disease.

Preliminary results of a review of sudden cardiac death in the adult with congenital heart disease were presented at the Canadian Adult Congenital Heart (CACH) Network meeting during the Canadian Cardiovascular Society's annual meeting in October 1994. Of 125 patients who were known to have died, sufficient details were available for 92 to determine the circumstance of death. Sudden death occurred in 23 patients (estimated incidence 5.3 per 1000 patients followed per year) at an average age of 33.5 +/- 11.9 years. Surgical procedures included intracardiac repair in 12, palliative procedures in only six and no cardiac surgery in six. Nine patients with sudden death had Eisenmenger syndrome. Right or left ventricular abnormalities were present in 15 of 21 patients with premorbid echocardiographic evaluation. A prior history of ventricular arrhythmia was available in only three patients. Sudden death is a significant cause of mortality in adults with congenital heart disease. Determination of risk factors will be an important aspect of the patient database under development by the CACH Network.

Adult↗
PubMed2004

Towards defining heart failure in adults with congenital heart disease.

Injury to the myocardium disrupts geometric integrity and results in changes to intracardiac pressure, wall stress and tension, and the pattern of blood flow through the heart. Significant disruption to pump function results in heart failure which is defined in terms of symptoms: breathlessness and fatigue, signs of salt and water retention, and neurohormonal activation. This syndrome most commonly occurs in the context of injury due to ischaemic heart disease and dilated cardiomyopathy but because patients with congenital heart disease (CHD) are born with sometimes gross distortions of cardiac anatomy they too are subject to the forces that drive heart failure. This paper explores the available data relating to the clinical and neurohormonal manifestations of heart failure in patients with congenital heart disease and describes how, by additionally exploring events at a cellular level, we may be able to arrive at a definition of heart failure relevant to this population.

Heart↗
PubMed1993

Orthotopic heart transplantation for congenital heart disease. Technical considerations.

Heart transplantation for congenital heart disease poses unique problems because of structural anomalies and previous corrective and palliative surgery. In the period between May 1985 and February 1992 a total of 231 orthotopic heart transplants were performed at our hospital -22 of these procedures were carried out in patients with congenital heart disease. The patient's ages ranged from 1 month to 51 years (median 10 years). There were 13 patients in the paediatric group (under 16 years) and 9 patients in the adult group (16 years or older). The diagnoses included univentricular connections (8 patients), complex morphology (4 patients), congenitally corrected transposition of the great arteries (TGA) (3 patients), hypoplastic left heart syndrome (2 patients), Fallot's tetralogy, tricuspid atresia, TGA, pulmonary atresia with intact ventricular septum and atrial septal defect (ASD) and ventricular septal defect (VSD). These patients had protected pulmonary circulation due to previous surgery or as a result of intrinsic pulmonary stenosis. Seventeen patients (77%) had undergone 29 prior operations (21 palliative and 8 corrective) including Blalock-Taussig shunts, pulmonary artery (PA) banding, ASD and VSD repair, Fontan procedure and Mustard operation. There were 2 early deaths in the paediatric group and 4 early deaths in the adult group, but no late deaths. The cause of death was multi-organ failure following uncontrollable haemorrhage in 3 patients, sepsis in 1 patient and donor organ failure in the 2 paediatric patients. Specific surgical manoeuvres were required to create the normal anatomical configuration. These included the rerouting of venous circulation, pulmonary artery reconstruction and atrial septation. Adequate donor tissue was taken to permit satisfactory reconstruction.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed2005

Color Doppler tissue imaging for evaluation of right ventricular systolic function in patients with congenital heart disease.

OBJECTIVE: We sought to explore the relationship of color Doppler tissue imaging-derived systolic indices of tricuspid valve annular motion and magnetic resonance imaging-derived right ventricular (RV) ejection fraction in patients with congenital heart disease. METHODS: Patients with congenital heart disease who underwent echocardiography and magnetic resonance imaging on the same day were included. The tricuspid valve annular color Doppler tissue imaging-derived parameters of peak velocity during isovolumic contraction, myocardial acceleration during isovolumic contraction, peak systolic velocity, and Tei index were compared with magnetic resonance imaging-derived RV ejection fraction. RESULTS: Peak systolic velocity and myocardial acceleration during isovolumic contraction correlated well with RV ejection fraction after adjusting for age, RV dilation, and pressure overload (r = 0.65 and 0.73, respectively). Interobserver and intraobserver reliability were excellent for peak systolic velocity (r = 0.95 and 0.97, respectively) and very good for myocardial acceleration during isovolumic contraction (r = 0.93 and 0.85, respectively). CONCLUSIONS: Color Doppler tissue imaging indices of tricuspid valve annular motion are reproducible and provide a potentially useful complementary tool for assessment of RV systolic function in patients with congenital heart disease.

Adolescent↗
PubMed2005

Congenital heart disease in relation to pulmonary hypertension in paediatric practice.

Pulmonary hypertension (PHT) is a well recognised feature of untreated congenital heart disease. This article will review the causes, known mechanisms, appropriate investigations and current therapies for PHT. The reader will understand the difference between PHT due to high pulmonary blood flow and PHT that is due to high pulmonary vascular resistance. The former is best treated by surgical or catheter intervention, whereas for the latter (Eisenmenger syndrome) only palliation is possible with medication or transplantation. Echocardiography and electrocardiography (ECG) should be performed in any child where there is a possibility of pulmonary hypertension, especially with long standing chronic lung disease and minor left to right shunt. Often these children may have dual pathology and their investigation and management may be a complex interaction between cardiac and respiratory therapists. New treatments and new techniques of assessment are now available and this may lead to improved recognition of PHT and prevention of long term disability as a result.

Child↗
PubMed1993

Modified Fontan procedure for complex congenital heart disease.

The Fontan procedure is frequently used for correction of complex congenital heart disease, but the postoperative morbidity and mortality rates are still high. The results in 35 consecutive patients who underwent the Fontan procedure from January 1980 to June 1991 are reviewed. The patients comprised 26 boys and nine girls aged from 1.7 to 14.8 (mean 6.8) years. The underlying diseases were univentricular heart (19 patients), tricuspid atresia (eight), complete atrioventricular canal (three patients, two of whom had straddling atrioventricular valve) and other conditions (five). Valved conduits were implanted in four patients and non-valved in 15. Direct right atrium-pulmonary artery anastomosis was performed in 16 patients. The overall hospital mortality rate was 23% (eight patients); in the final year of the review it was 11%. Follow-up of the survivors ranged from 1 to 120 (mean 25) months. All except two patients attained New York Heart Association functional class I or II. Three patients underwent reoperation for atrioventricular valve regurgitation and conduit stenosis with good results. It is concluded that the Fontan procedure is a good palliative operation for complex congenital heart disease.

Adolescent↗