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PubMed1993

Two-stage sternal closure after repair of congenital heart disease in neonates.

Closure of the longitudinal sternal incision can lead to unacceptable impairment of the haemodynamic state after prolonged operation for complex congenital heart disease associated with decreased cardiac function which is caused by cardiac dilatation. Between 1981 and 1991, a two-stage sternal closure was used in 21 neonates with dilatation of the heart and low cardiac output after correction of congenital heart disease. The mean age at operation was 14 (range 4-30) days. In 15 patients (group 1), primary closure of the sternum was considered impossible in the operating room; in the remaining six (group 2), the sternum was reopened on the day of operation or on the first or second day after repair. Three of these newborn infants had a total anomalous pulmonary venous connection and 18 a simple transposition of the great arteries. In all patients, temporary closure of the thorax with a dura mater patch was performed. The patch was usually removed on day 4 after operation in the intensive care unit and followed by uncomplicated routine chest closure. There were no problems with mediastinitis, wound infection, osteomyelitis or instability of the sternum. Subsequent deformity of the thorax was not observed. Some 11 of 15 children (73%) of group 1 and four of six (66%) of group 2 survived. Leaving the sternum open resulted in a significant improvement in the haemodynamics in all patients. The technique of two-stage closure of the sternum is very effective after lengthy corrective operation in children with congenital malformations of the heart who require long periods of perfusion or ischaemia.

Cardiac Output, Low↗
PubMed1982

Two-dimensional echocardiographic assessment of right ventricular volume in children with congenital heart disease.

The ability of 2-dimensional echocardiography to measure right ventricular (RV) volume and ejection fraction was assessed in 22 children with congenital heart disease. From the apical 4 chambers 2-dimensional echocardiographic image, the long-axis length of the right ventricle was measured and the area planimetered. On the anteroposterior and lateral cineangiocardiographic planes, the right ventricle was separated into 2 parts: RV sinus and outflow tract. The longest length, inflow tract length, and area of the sinus were measured from biplane cineangiographic views. The echographic long-axis length correlated well with the longest length of the RV sinus measured from both anteroposterior and lateral cineangiographic views at both end-systole and end-diastole. Moreover, the echographic area correlated well with the sinus area obtained from both cineangiographic views. From these regression analyses, the echographic long axis length and area were corrected to the angiographic longest length and area of the sinus. The new corrected echographic longest length and area were applied to 3 formulas (2 biplane and 1 uniplane) to calculate the sinus volume of the right ventricle. Total RV volume was then derived from the sinus volume. RV volumes and ejection fraction determined by 2-dimensional echocardiography were compared with those obtained from biplane cineangiography using Simpson's rule method. All formulas tested predicted RV volumes and ejection fraction with equal accuracy. Thus, 2-dimensional echocardiography can assess RV volume and ejection fraction in children with congenital heart disease.

Angiocardiography↗
PubMed1985

Peripheral neuroblastic tumors and congenital heart disease. Possible role of hypoxic states in tumor induction.

Peripheral neuroblastic tumors (PNT), including neuroblastomas, pheochromocytomas, ganglioneuromas, and paragangliomas, have been reported in association with congenital heart disease (CHD). If a significant correlation between PNT and CHD could be demonstrated, it would suggest that peripheral neuroblastic tumors might represent reactive, secondary phenomena rather than independent or incidental events. We examined this question by reviewing the autopsy files of The Johns Hopkins Hospital and found that the frequency of congenital cardiovascular problems among patients with PNT was significantly greater than the frequency of CHD in the general autopsy population (p less than 0.001). There was no correlation between CHD and central neuroblastic tumors (CNT), i.e., medulloblastomas, retinoblastomas, or pinealoblastomas, or between CHD and metastatic malignant melanoma. We examined the hypothesis that erythropoietic activity, as might be stimulated by hypoxia, was correlated with the presence of peripheral neuroblastic tumors. We found that most (87%) cardiac malformations associated with PNT produced cyanosis, and histologic assessment of hematopoiesis revealed greater activity in patients with PNT (with or without CHD) compared to those with CNT (p less than 0.05) or malignant melanoma (p less than 0.05). In addition, patients with PNT and CHD had higher frequencies of extramedullary hematopoiesis compared to age-matched controls with similar congenital heart defects (p less than 0.05), and compared to patients with CNT or metastatic malignant melanoma (p less than 0.01). These results suggest that peripheral neuroblastic tumors may arise in a setting of chronic hypoxia produced by congenital heart disease.

Adrenal Gland Neoplasms↗
PubMed1998

Surgery for congenital heart disease in Europe 1995.

OBJECTIVE: An attempt was made to find out how many operations were performed in Europe in 1995 for congenital heart disease. METHODS: Representatives from the 20 most active European countries which could supply reliable information were contacted. They were asked to contact all surgeons performing significant numbers of operations for congenital heart disease in their country. The numbers of operations on children under and over 1 year operated on with and without cardiopulmonary bypass were collected. No attempt was made to collect more detailed information such as operative mortality as it was felt that many surgeons would be reluctant to give their results and the data would be incomplete. RESULTS: A total of 27,976 operations were recorded, of which 20,318 were performed with cardiopulmonary bypass and 7658 without. The total population of the 20 European countries was 448 million and the mean number of operations performed with cardiopulmonary bypass per million was 45.4. However, the rate varied from 9.1 to 70.1. The percentage of operations on cardiopulmonary bypass performed in the first year of life varied from 9.4% to 44.4%. CONCLUSIONS: The total of nearly 28,000 operations in 1995 is probably an underestimate, but it has been difficult to collect this data at all. Obviously many smaller countries have not been included, and some older patients undergoing operations such as atrial septal defect closure in adult units have been missed. The number of open heart operations per million vary in different countries and this presumably represents differing referral patterns. If their numbers are low in some countries, these results may be helpful in persuading their governments that more resources are needed for congenital heart surgery. It is also interesting to see the variation in the number of operations performed in the first year of life. Those countries with a lower rate may have a backlog of older patients who were previously palliated, or their surgeons may still prefer initial palliation and later correction in some conditions rather than early correction. It is hoped that with better prospective data collection, an assessment can be repeated in 2000.

Cardiac Surgical Procedures↗
PubMed1995

Transthoracic three-dimensional echocardiography in adult patients with congenital heart disease.

OBJECTIVES: This study sought to assess both the feasibility and potential role of transthoracic three-dimensional echocardiography for the evaluation of adult patients with congenital heart disease. BACKGROUND: The unrestricted views with depth perception provided by three-dimensional echocardiography with dynamic volume-rendered display may enhance visualization of cardiac structures and detection of abnormalities in patients with congenital heart defects. METHODS: We studied 33 patients with various heart defects (mitral valve anomalies in 9, aortic valve anomalies in 5, subaortic membrane in 5, ventricular septal defect in 4, transposition of the great arteries in 3, tetralogy of Fallot in 2, other defects in 5). Cross-sectional images of the specific region of interest were acquired from either the parasternal or apical window with the rotational technique (2 degrees interval with electrocardiographic and respiratory gating) and postprocessed for resampling in cubic format. From these three-dimensional data sets a multitude of cut planes were selected, presented in volume-rendered dynamic display and analyzed by two observers for comparison with standard two-dimensional images to assess their additional information. RESULTS: Three-dimensional reconstruction was possible in all patients. Structures of interest were evaluated from unusual viewpoints, providing both cardiologists and surgeons with immediate feedback. When compared with standard two-dimensional images, additional information was provided for 12 patients (36%). The mitral valve, aortoseptal continuity and interatrial septum were the structures for which three-dimensional echocardiography was most useful. CONCLUSIONS: Transthoracic three-dimensional echocardiography is feasible and facilitates spatial recognition of the intracardiac anatomy in a significant proportion of patients and enhances diagnostic confidence of complex congenital heart disease.

Adolescent↗
PubMed2006

Impact of cardiac surgery on plasma levels of B-type natriuretic peptide in children with congenital heart disease.

OBJECTIVE: To examine prospectively the effect of cardiac surgery on plasma concentration of B-type natriuretic peptide (BNP) in children with congenital heart disease. SETTING: Tertiary referral center. METHODS: BNP plasma concentration was measured by sandwich immunoassay (Biosite) in 65 consecutive patients with congenital heart disease (age 4 days to 17.1 years, mean (S.D.) age 3.6 (4.7) years, median age 1.0 years) on the day before and after surgical therapy. BNP levels were compared to perioperative data and to healthy subjects. RESULTS: BNP increased significantly (p<0.001) after cardiac surgery from median 31 pg/ml (mean 189 pg/ml) to median 453 pg/ml (mean 607 pg/ml) and remained significantly elevated during the first week (p<0.001). After a first peak mean (S.D.) 1.3 (0.7) days after surgery, there was a significant decrease of plasma BNP followed by a second peak 5.1 (1.1) days after surgery. Postoperative BNP plasma concentration was correlated to BNP before surgery (r=0.58, p<0.001), to cardiopulmonary bypass duration (r=0.52, p<0.001) and to serum lactate concentration at the first day after surgery (r=0.49, p<0.001). CONCLUSIONS: In children with congenital heart defects plasma BNP increased immediately after cardiac surgery despite haemodynamic unloading. The correlation to cardiopulmonary bypass time and serum lactate concentration and the similarity between the pattern of BNP release after surgery and after myocardial ischaemia might be explained by a cytoprotective role for BNP after cardiac surgery.

Adolescent↗
PubMed2000

Effect of physical training in children and adolescents with congenital heart disease.

In order to test the effect of systematic supervised physical training, we divided a total of 129 children and adolescents with congenital heart disease into a group undergoing intervention and a control group. All patients underwent exercise tests, measurements of physical activity, and a survey of psychosocial factors. An improvement in uptake of peak level of oxygen was observed after intervention. There was also an improvement in physical activity in both groups measured by a monitor, although this was significant only in those with intervention. The psychosocial scales measured by the Child Behavior Checklist showed a decrease in internalizing scores for those subjected to intervention. This was decreased due to decreased withdrawal and somatic complaints. In conclusion, we recommend systematic supervised training, including testing of routine follow-ups, in patients with congenital heart disease.

Analysis of Variance↗
PubMed1986

The role of cross sectional echocardiography and pulsed Doppler ultrasound in the management of neonates in whom congenital heart disease is suspected. A prospective study.

The application of cross sectional echocardiography and pulsed Doppler ultrasound to the management of symptomatic neonates with suspected congenital heart disease was studied in 96 consecutive cases. The ability of echocardiography to establish a complete and accurate diagnosis and a correct management plan was evaluated. Sequential segmental analysis of data from cardiac catheterisation and necropsy identified 536 cardiovascular anomalies. Of the 536 anomalies 512 were correctly diagnosed by echocardiography (sensitivity 95.5%). Seven false positive echocardiographic diagnoses were made (specificity 98.6%). Nearly all the missing diagnoses and all the false positive diagnoses made by echocardiography were extracardiac vascular anomalies. Normal cardiovascular anatomy was at all times correctly identified by echocardiography. In 12 babies (12.5%) a management plan could not be established because of inconclusive echocardiographic findings. Of the 84 proposed plans based on the echocardiographic findings, eight were found to be inappropriate after catheterisation. Thus, 76 babies (79.2%) could have been correctly managed without cardiac catheterisation. The combination of cross sectional echocardiography and pulsed Doppler ultrasound not only allows diagnosis of congenital cardiac anomalies in most neonates but can facilitate appropriate clinical management. Only a minority of neonates with suspected congenital heart disease require cardiac catheterisation and angiography.

Aortography↗
PubMed1998

Cardiorespiratory response during exercise in patients with cyanotic congenital heart disease with and without a Fontan operation and in patients with congestive heart failure.

To clarify the different cardiorespiratory response to exercise in patients with congenital heart disease and patients with chronic congestive heart failure, we investigated the effect of a progressive exercise test in 30 patients aged 10 to 24 years, including 9 patients with cyanotic congenital heart disease (group A), 13 patients who had undergone a Fontan operation (group B), and 8 patients with reduced left ventricular function (group C), and 18 healthy controls (group D). There was no difference in peak oxygen uptake among patient groups and all group A, B, and C values were lower than those in group D (P<0.001). Although peak heart rate was lower in patient groups than in group D, heart rate at a given exercise intensity was highest in group C. The oxygen pulse (oxygen uptake divided by heart rate=stroke volume x arterial venous oxygen difference), as an indicator of stroke volume, was lower in patients groups, especially in group C, than in group D. There was no difference in tidal volume between groups A and D, but the respiratory rates at any given exercise intensity were higher in group A than in the other patient groups, thus minute ventilation and the ventilatory equivalent were highest in group A. The increased respiratory rate and low tidal volume in group C resulted in rapid and shallow respiration. There was no difference in exertional symptoms at peak exercise among the groups. In addition to impaired responses of stroke volume during exercise in patients with reduced exercise capacity, there was little limitation of increase in ventilation in group B and excessive ventilation in group A. The present results suggest that relationship between ventilatory and cardiac responses during exercise in patients with cyanotic congenital heart disease with and without a Fontan operation is different from the relationship in patients with chronic congestive heart failure; however, these pathological differences did not influence exertional symptoms.

Adolescent↗
PubMed2006

Ablation of nonautomatic focal atrial tachycardia in children and adults with congenital heart disease.

INTRODUCTION: Nonautomatic focal atrial tachycardia (NAFAT) has been characterized in adults with structurally normal hearts. This article characterizes NAFAT in a population of patients with complex congenital heart disease. METHODS AND RESULTS: Electrophysiologic and electroanatomic mapping data and acute outcomes were reviewed in patients undergoing mapping and ablative procedures for atrial tachycardia at Children's Hospital, Boston, between January 1999 and December 2003. Twenty-two NAFAT foci were identified in 17 patients out of 216 patients studied. Fourteen of these 17 patients had congenital heart disease. The average age of the patients with a NAFAT mechanism was 27 years and there was no gender predilection. The presumptive diagnosis based on clinical grounds and surface ECG assessment in 11 of 17 patients with NAFAT was atrial flutter. None of the 17 patients were suspected of having a NAFAT mechanism by noninvasive assessment. Four of the 10 patients had both NAFAT and macroreentrant atrial tachycardias. NAFAT cycle lengths varied widely (200-680 ms) between patients. Sixteen of the 22 NAFAT foci were mapped to the anatomic right atrium (RA). Acute ablative success was achieved in 17 out of 22 foci (77%). CONCLUSION: NAFAT is relatively uncommon in a pediatric tertiary care setting, and in that setting occurs most often in adults with congenital heart disease. NAFAT is indistinguishable from other forms of atrial tachycardia by noninvasive means and can mimic other forms of atrial tachycardia on electrocardiogram. The foci were predominantly found in the RA and were, in most cases, acutely amenable to catheter ablation therapy.

Adolescent↗
PubMed2005

Somatostatin for postoperative chylothorax after surgery for children with congenital heart disease.

Chylothorax is a rare but serious postoperative condition with a high rate of morbidity that may lead to death of children with congenital heart disease. Here we reviewed nine consecutive cases with chylothorax in infants and children following cardiac surgery from March 2002 to February 2003. Somatostatin was added to conservative treatment protocol to increase effectiveness of therapy in all cases. The duration of somatostatin therapy varied from 7 to 32 days. All cases of chylothorax were successfully treated with intravenous infusion of somatostatin as an adjunctive treatment. Even though two cases showed rebound phenomena, we avoided any surgical procedure in the nine patients who treated with conservative management combined with somatostatin. No significant side effects of somatostatin were observed. It seems that somatostatin is effective, noninvasive and safe therapeutic modality. It can be used as an adjunctive treatment to conservative management to control postoperative chylothorax in children with congenital heart disease.

Child, Preschool↗
PubMed2003

Plasma leptin levels in children with cyanotic and acyanotic congenital heart disease and correlations with growth parameters.

BACKGROUND: Leptin has been shown to be an integral component of energy homeostasis and regulation of body weight. Leptin regulates adipose tissue mass and correlates with the fat mass, however the circulating levels are altered by energy intake. Research on the physiological function of leptin has primarily focused on its role in the pathogenesis of obesity. However, its role in the negative energy imbalance is unclear. Increased energy expenditure is a primary factor in the reduced growth in infants with cyanotic congenital heart disease. The objective of this study was to examine the possible role of leptin on growth and nutrition in children with cyanotic and acyanotic congenital heart disease. METHODS AND RESULTS: In this study, plasma leptin levels, nutritional and growth status were evaluated in 28 cyanotic and 20 acyanotic patients with congenital heart disease. Although standard deviation (S.D.) of height (P<0.01), mid arm circumference (MAC) (P<0.001) and body mass index (BMI) (P<0.05) were significantly low in cyanotic group, plasma leptin levels were similar. Energy intake was high in cyanotic group. In both cyanotic and acyanotic group, plasma leptin levels were correlated with BMI (R: 0.388, P<0.05 and R: 0.789, P<0.001, respectively). In addition, leptin levels were significantly correlated with the height (R: 0.415, P<0.05), MAC (R: 0.482, P<0.05) and BMI (R: 0.377, P<0.05) S.D. in cyanotic subjects. CONCLUSIONS: Our results suggest that the leptin regulating axis is intact in cyanotic patients and leptin does not contribute to the cachexia of cyanotic heart disease.

Adolescent↗
PubMed1997

Permanent overdrive atrial pacing in the chronic management of recurrent postoperative atrial reentrant tachycardia in patients with complex congenital heart disease.

This study investigated the value of permanent atrial pacing as an adjunct to the current therapy in the chronic management of recurrent postoperative atrial reentrant tachycardia in patients with complex congenital heart disease. We studied the postpacing clinical course in 18 patients with recurrent atrial reentrant tachycardias unresponsive to conventional therapy who had an implanted atrial pacemaker. The pacemaker was programmed at a lower pacing rate 20% faster than the spontaneous mean daily rate previously determined with 24-hour Holter monitoring. Serial Holter recordings and pacemaker programming sessions were subsequently performed trying to maintain a paced atrial rhythm overdriving the spontaneous rhythm as long as possible. Twenty-four hour Holter monitoring documented a prevalent (> 80%) paced rhythm during the daily hours in all patients during the follow-up; all patients, however, required at least once a variation in programmed mode and pacing rate. Antiarrhythmic medications were discontinued after 6 months if the patient remained arrhythmia free while on pacing. Recurrences of atrial reentrant tachycardia occurred in five patients (29%) during the initial 6 months interval after the pacemaker implantation, while late recurrences occurred in only two patients (11%). One patient died suddenly 10 months after the pacemaker implant. At the end of the follow-up, 15 patients (83%) were arrhythmia-free and only 2 of them were still on antiarrhythmic drugs. We conclude that permanent atrial overdrive pacing can be an important tool in the management of patients with atrial reentrant tachycardia following repair of congenital heart disease.

Adolescent↗
PubMed1982

congenital heart diseases in swine.

One hundred twenty-two congenital cardiac anomalies were diagnosed in 83 pigs (4.35%) during necropsies of 1906 pigs from one day to four years of age during an 11-month period. The incidence of cardiac malformation was higher at 29 to 56 days of age. Of the 83 pigs with cardiac anomalies, 47 (56%) were male and 36 (44%) were female, and of these, 15 (18%) were purebred and 68 (82%) were crossbred pigs. Of the 15 purebred pigs with cardiac malformation, there were seven Landrace pigs, four Large White pigs, three Duroc Jersey pigs, and one Yorkshire pig; whereas 94% of the 68 crossbred pigs were crossbred among Landrace, Duroc Jersey, and Large White. The 122 cardiac anomalies found in the 83 pigs were: dysplasia of the tricuspid valve in 42 pigs, atrial septal defect in 31 pigs, subaortic stenosis in 22 pigs, ventricular septal defect in nine pigs, persistent common atrioventricular canal in eight pigs, malformation of the moderator band in seven pigs, persistent vena cava in one pig, persistent truncus arteriosus in one pig, and pulmonary stenosis in one pig. The pathological features of the anomalies in swine were similar to those described in small animals and in man. The findings indicate that spontaneous porcine cardiac anomalies might provide models for cardiovascular investigators to study the etiology and pathogenesis of congenital heart diseases in man and other animals.

Animals↗
PubMed2003

Pericardial effusion after open heart surgery for congenital heart disease.

OBJECTIVES: To determine the prevalence and time course of pericardial effusion after open heart surgery for congenital heart diseases and to identify predisposing risk factors. DESIGN AND PATIENTS: Prospective assessment of development of pericardial effusion in 336 patients (163 males) undergoing open heart surgery for congenital heart disease by serial echocardiography on days 5, 7, 14, 21, and 28 postoperatively. SETTING: Tertiary paediatric cardiac centre. RESULTS: The prevalence of pericardial effusion was 23% (77 of 336). Of the 77 patients who developed effusion, 43 (56%) had moderate to large effusions and 18 (23%) were symptomatic. Patients who had a large amount of effusion were more likely to be symptomatic than those with only a small to moderate amount (47.4% v 15.5%, p = 0.01). The mean (SD) onset of pericardial effusion was 11 (7) days after surgery, with 97% (75 of 77) of cases being diagnosed on or before day 28 after surgery. The prevalence of effusion after Fontan-type procedures (60%, 6 of 10) was significantly higher than that after other types of cardiac surgery: repair of left to right shunts (22.1%, 43 of 195), repair of lesions with right ventricular outflow tract obstruction (22.6%, 19 of 84), arterial switch operation (6.7%, 1 of 15), and miscellaneous procedures (25%, 8 of 32) (p = 0.037). Univariate analyses showed that female patients (p = 0.009) and those receiving warfarin (p = 0.002) had increased risk of postoperative pericardial effusion. A greater pericardial drain output in the first four hours after surgery also tended to be significant (p = 0.056). Multivariate logistic regression similarly identified warfarin treatment (beta = 1.73, p = 0.009) and female sex (beta for male = -0.63, p = 0.037) as significant determinants. CONCLUSIONS: Pericardial effusion occurs commonly after open heart surgery for congenital heart disease. Serial echocardiographic monitoring up to 28 days postoperatively is indicated in selected high risk patients such as those with symptoms of postpericardiotomy syndrome and those given warfarin.

Analysis of Variance↗
PubMed2003

Methemoglobin formation in children with congenital heart disease treated with inhaled nitric oxide after cardiac surgery.

OBJECTIVE: Inhaled nitric oxide (NO) is used as a therapy of pulmonary hypertension in children after cardiac surgery. Hemoglobin binds to NO with great affinity and forms methemoglobin by oxidation in the erythrocyte. Once produced, methemoglobin is unable to transport and unload oxygen in the tissues. The amount of available hemoglobin in the body for oxygen transport is thereby reduced. Anemia, acidosis, respiratory compromise and cardiac disease may render patients more susceptible than expected for a given methemoglobin level. The goal of the present study was to review the cumulative effect of inhaled NO on methemoglobin formation in critically ill children. We therefore looked for methemoglobin levels in children with congenital heart disease after cardiac surgery who were treated with inhaled NO in a range of 5-40 ppm. METHODS: We retrospectively reviewed the medical charts of 38 children with congenital heart disease after cardiac surgery. We extracted demographic data and physiological measurements at the following time points: (1) T0 = before starting inhaled NO therapy, (2) T1 = 24 h after the beginning of inhaled NO therapy, (3) T2 = half-time therapy, (4) T3 = end of therapy, (5) T4 = 24 h after finishing inhaled NO therapy. RESULTS: The median duration of inhaled NO therapy was 5.5 days (interquartile range 6, range 2-29), NO concentrations at T1 and T2 were 16 ppm (10, 5-40) and 12.5 ppm (12.3, 2-40), respectively. The median cumulative dose of inhaled NO was 1699 ppm (2313, 193-7018). Methemoglobin levels increased moderately, but significantly, during therapy ( T0 vs T1 p<0.05 and T0 vs T2 p<0.001). The highest methemoglobin level measured was 3.9%. Methemoglobin levels correlated positively with the inhaled NO doses applied at T1 ( r(2)=0.8376; p<0.01) and at T2 ( r(2)=0.8945; p<0.01). At T1 the methemoglobin level correlated negatively with the T1 blood pH value. The overall mortality rate was 13.2% (5 of 38 study patients died). There was no significant difference in methemoglobin levels between survivors and non-survivors. CONCLUSION: We conclude from our data that the use of inhaled NO therapy for children with congenital heart disease after cardiac surgery in the described range of 5-40 ppm, resulting in a maximum of 4% methemoglobin blood level, is feasible and safe. However, we recommend the use of the minimal effective dose of inhaled NO and continuous monitoring of methemoglobin levels, especially in cases of anemia or sepsis in critically ill children.

Administration, Inhalation↗
PubMed1990

'Grown-up' survivors of congenital heart disease: who knows? Who cares?

'Grown-up' congenital cardiac patients are an increasing medical community, not at present being properly cared for in any country. Information on over 2000 patients aged 15-65 years with congenital heart disease who have been treated in the National Heart Hospital provides the data for this report.

Activities of Daily Living↗
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↗