Grown-up Congenital Heart Patients Research Articles

Quality of life, psychological resilience, personality traits and illness perception in grown-up congenital heart patients in Hong Kong

BackgroundStudies suggested heterogeneity of physical and psychological predictors of quality of life (QOL) among congenital heart disease patients. This cross-sectional study aimed to examine the interrelationships of QOL, health status, resilience, personality traits, illness perception, and clinical data of grown-up congenital heart (GUCH) patients in a tertiary center in Hong Kong. MethodsGUCH patients (N ​= ​208, male ​= ​103) were invited to complete self-report questionnaires: 36-item Short Form Health Survey version 2 (SF36), Satisfaction with Life Scale (SWLS), Connor-Davidson Resilience Scale (RISC), Hospital Anxiety and Depression Scale (HADS), and NEO Five Factor Inventory (NEOFFI-3); results on these measures were compared to 104 healthy peers. GUCH patients also completed Brief Illness Perception Questionnaire (BIPQ). Disease complexity, illness severity, New York Heart Association (NYHA) class and Warnes-Somerville Ability Index (WSAI) grades of patients were obtained. The interrelationships between these patient-reported outcome (PRO) measures, clinical indices and functional status were assessed. ResultsQOL scores and health status were lower in GUCH patients than healthy peers, and were associated with greater disease severity and worse functional class (all p-values < 0.01). SWLS and health status correlated positively with RISC, but negatively with HADS and negative illness perception (all p ​< ​0.001). In multivariate regression, lower RISC, negative illness perception, and greater neuroticism were predictors of lower SWLS (all p ​< ​0.05); higher WSAI grade, lower RISC and negative illness perception were predictors of lower PCS (all p ​< ​0.05); and greater neuroticism and HADS were predictors of lower MCS (all p ​< ​0.01). ConclusionsReduced QOL and health status among GUCH patients were associated with both clinical and psychological attributes. Resilience and illness perception might be the potential targets of intervention.

Non-invasive isthmus identification of complex arrhythmias in congenital heart disease.

Sustained re-entrant tachyarrhythmias treatment has become pivotal in the grown-up congenital heart patients clinical management. Cardiac LGE-MRI tissue characterization integrated with high definition electroanatomic map could allow fast recognition and effective treatment of substrate of tachyarrhythmias. Cardiac LGE-MRI areas were suggestive of post-surgical changes both in atrium and ventricle. High-density electro-anatomical map localized areas of slow conduction identifying conducting isthmuses of re-entrant arrhythmias.

Strategy of Medical Care for Pregnant Women with Congenital Heart Disease

Grown-up congenital heart (GUCH) is a global challenge nowadays. The strategy of medical care for GUCH women dur-ing pregnancy, childbirth and the postpartum period is a topic of active discussion in the expert community. These patients have significantly increased risk of maternal and perinatal loss. A national obstetric cardiology and cardiac surgery multi-disciplinary team (OCCS) established in academic institutions in 2013 has provided medical support to 896 GUCH pregnant women over the last 7 years. In total, GUCH patients accounted for 36% of the cohort of all the examined pregnant women. Of these, 474 (53%) were primigravid. The mean age of the patients at the time of the first visit was 27.3 ± 5.7 years. Patients with uncorrected CHD accounted for 66.2% (n = 593), and 33.8% (n = 303) of pregnant women had undergone correction, including hemodynamic correction of complex CHD in 5 patients. Risk stratification was performed using several scores (mWHO, ZAHARA, CARPREG) for the comprehensive assessment of cardiovascular risk and prediction of pregnancy, deliv-ery, and postpartum period course. 82 patients were classified as having high cardiovascular risk (CVR) after the stratifica-tion. They needed admission to the cardiac surgery facility to receive different types of medical care. There were 2 (2.4%) cases of maternal loss and 3 (3.8%) cases of adverse perinatal outcomes in this group of patients. The article presents the algorithms for multidisciplinary care strategy choice in GUCH pregnant women with high CVR and their routing principles developed by the OCCS. These algorithms significantly reduced adverse outcomes of pregnancy and childbirth in this group of patients. Long-term results were evaluated in 69 patients (86.3%). The follow-up period ranged from 1 to 91 months, on average 34.4 ± 23.6 months. There were no long-term maternal losses or repeated cardiac surgeries. There was one case of unexplained death of a child 8 months after birth. The strategy of multidisciplinary medical care of a high-class GUCH pregnant woman should be personalized depending on the clinical data and in accordance with the ESC 2018 guidelines.

Impact of the perioperative inotropic support in grown-up congenital heart patients undergoing cardiac surgery: a propensity score adjusted analysis

BackgroundGrown-up congenital heart (GUCH) patients represent a growing population with a high morbidity risk when undergoing reparative surgery. A main preoperative feature is right ventricular failure, which represents a risk factor for postoperative low cardiac output syndrome. Levosimendan has a potentially beneficial effect. This retrospective study included consecutive GUCH patients with surgeries in a tertiary cardiothoracic centre between 01-01-2013 and 01-10-2017, to test the hypothesis that the postoperative use of levosimendan might be associated with shorter time of mechanical ventilation, when compared with the use of milrinone. To adjust for bias related to the probability of treatment assignment, it uses the inverse propensity score weighting methodology.ResultsOverall 363 patients had GUCH surgeries during the study period, their mean age was 31.39 ± 15.31 years, 87 patients were eligible for analysis in the Levosimendan group and 117 in the Milrinone group. The propensity score used pre- and intraoperative variables and resulted in a good balance between covariates. The Levosimendan group included patients with higher preoperative risk scores, a higher prevalence of left and right ventricular failure, who required more often the addition of epinephrine, renal replacement therapy, prolonged mechanical ventilation and intensive care stay. However, after propensity score weighting, patients in the Levosimendan group had shorter durations of mechanical ventilation (average treatment effect − 37.59 h IQR [− 138.85 to − 19.13], p = 0.01) and intensive care stay (average treatment effect − 3.11 days IQR [− 10.03 to − 1.48], p = 0.009). The number of days of additional epinephrine support was shorter and the vasoactive inotropic scores lower.ConclusionWe report a beneficial effect in terms of duration of mechanical ventilation and intensive care stay, and on inotropic requirements of the use of levosimendan following GUCH surgeries. The use of levosimendan in this setting requires validation at a larger scale.

P4695Pulmonary hypertension in grown-up congenital heart disease, 2018 Nice classification. Impact on mortality. GUCH Registry data

Abstract Background Pulmonary hypertension (PH) is a serious complication of grown-up congenital heart (GUCH) diseases. Its onset is associated with a marked increase in mortality in these patients. Purpose To classify GUCH patients with pulmonary hypertension (PH) according to PH subgroups, and explore association between PH and mortality during follow up. Methods An observational retrospective study was conducted on patients on the GUCH database. Overall data were analyzed. Patients diagnosed with PH were selected and classified according to the 2018 Nice HT guidelines on pulmonary arterial hypertension secondary to congenital heart disease: Group 1 (PAH1) and its 4 subgroups (SG1: Eisenmenger's syndrome; SG2: left to right shunts; SG3: PAH associated with congenital heart disease; SG4: post-operative PAH); Group 2 (PH2), Group 3 (PH3), Group 4 (PH4), Group 5 (PH5). Statistical analysis: Kaplan Meier Log Rank test using SPSS 20 software. Results The database included 2073 patients (P), mean age 31.8±12.9 years, 54.2% (1123 P) were women. Overall mortality: 4.2% (88 P). Follow up: 44.1±37 months. Results showed 5.1% of patients (105 P) had PH, 3.8% (79 P) of whom corresponded to group PAH1, and 1.3% (26 P) to the remaining groups. Analysis of P with PH showed that 67.6% (71 P) were women, mean age was 41.3±14.7 years, mortality during follow up was 25.7% (27 P). Classification according to group showed 75.2% (79) corresponded to PAHG1, 9.5% (10) to PH2, 0% to PH3, 1% (1) to PH4, and 14.3 (15) to PH5. Analysis of P in PAHG1 showed a 43% (34 P) prevalence of SG1, 27.8% (22 P) of SG2, 8.9% (7 P) of SG3, and 20.3% (16 P) of SG4. Mortality per group was 22.8% (18 P) in PAH1, 20% (2 P)in PH2, 0% in PH 3, 0% in PH 4, and 46.7% (7 P) in PH 5. Kaplan Meier survival curves showed significant association between PH and mortality, Log Rank=0.000, and worst survival in groups PH2, PAH1 and PH5. No differences in mortality were observed among PAH1 subgroups, Log Rank= 0.458. Survival curves Conclusions PH negatively affected survival of GUCHpatients. Analysis of mortality during follow up showed patients in group PH5 to have the worst outcomes, possible because patients with a single ventricle were included in this group. Group PAH1 comprised 75% of PH patients, with a greater predominance of patients with Eisenmenger's syndrome, but no significant differences in mortality during follow-up among the 4 subgroups.

Pattern of un-operated Grown Up Congenital Heart (GUCH) patients presenting to a Tertiary Care Cardiac Institute of Punjab.

Objectives:To identify the pattern of un-operated grown up congenital heart defects at our tertiary care cardiac institute.Methods:This is a prospective observational study. All un-operated GUCH patients who presented to Faisalabad Institute of Cardiology (FIC) from May 2017 to 30th July 2017 were enrolled. Diagnosis was established on Transthoracic Echocardiography done by dedicated pediatric cardiologist at FIC. The mode of presentation, presenting complaints, type, severity, complications and co-morbid conditions of CHD were recorded.Results:A total of 200 consecutive patients were enrolled. Mean age was 29.92 ± 11.21 years. There were 104 females (52%) and 96 males (48%). Majority of patients presented in Out-Patient Department (84%) while 16% presented in emergency (n=32). The most common cardiac anomalies were: Atrial Septal Defect (ASD) 41.5% (83), Tetralogy of Fallots (TOF) 42 (21%), Ventricular Septal Defect (VSD) 28 (14%) and Patent ductus arteriosus (PDA) 8 % (16). Cyanotic CHD was present in 43% (86) while TOF was the most common of it. The disease was of moderate complexity in 77.5% patients. Certain complications like Pulmonary hypertension 69(34.5%), Eisenmenger 33(16.5%), Rhythm disturbances 15 (7.5%), Infective endocarditis 5(2.5%) were also present along with co-morbid conditions like coronary artery disease (1.5% and systemic hypertension (2.5%). Dyspnea on exertion (59.5%) followed by cyanosis (41%) were the most common presenting complaints. The most common reason for hospital admission was cardiac signs and symptoms (19.5%) followed by cardiac catheterization (10.5%).Conclusion:The ASD, TOF, VSD and PDA remain the most common CHD in descending order while pulmonary hypertension, Eisenmenger, heart failure, arrhythmias, infective endocarditis and stroke were the common complications of CHD at this particular age.

A Novel Designed Valved Conduit for RVOT Reconstruction in Grown-up Congenital Heart Patients: a Glimpse Down the Road.

A plethora of valves and valve conduits are available for reconstruction of the right ventricular outflow tract (RVOT) for grown-up congenital heart patients. However, for several reasons, the ideal pulmonary valve substitute still remains the subject of debate. In this study, we investigated the preliminary clinical and echocardiographic results after implantation of the RVOT Elan (Vascutek, Renfrewshire, United Kingdom) conduit in adolescents and adults. Between October 2012 and December 2014, a total of 27 patients (19 males, mean age: 23.7 ± 22.5; range: 9-74 years) received a RVOT Elan conduit for RVOT reconstruction and were prospectively followed up clinically and echocardiographically. Twenty-five patients had previous cardiac surgery. The median number of prior operations per patient was 2 (range: 1-4). Tetralogy of Fallot was the most common diagnosis (n = 7). At a mean follow-up time of 0.9 ± 0.61 years (100% complete), all patients (27 of 27) were alive and in New York Heart Association Class I. Adverse events defined as valve failure, thrombosis, embolism, bleeding, or endocarditis did not occur. Freedom from reoperation in general was 100%. At 1-year follow-up, median peak pressure gradients (Δ Pmax) across the RVOT Elan conduit were 15 ± 3.2; 15.3 ± 2.1Δ, 16 ± 4.8, and 16.3 ± 5.1 mm Hg for the 19 (n = 3), 21 (n = 3), 23 (n = 6), and 25 mm (n = 15) conduit size, respectively. The RVOT Elan conduit revealed excellent preliminary clinical and hemodynamic performances independent from the underlying cardiac pathology with insignificant transvalvular gradients and nonturbulent flow characteristics.

Life with congenital heart disease and pulmonary hypertension

The Grown-Up Congenital Heart Patients’ Association (GUCH-PA), Pulmonary Hypertension Association and Downs Heart Group are jointly looking into patients’ experiences of services for those with a dual diagnosis, such as Hayley Jackson, who shares her story here.

Recurrent Pneumonia and a Normal Heart: Late Complication after Repair of Hemianomalous Pulmonary Venous Drainage—A Cautionary Tale

Hemianomalous pulmonary venous drainage with intact atrial septum is a rare congenital anomaly and reports of its surgical repair and the long-term complications related to the correction are only infrequently encountered in the literature. We report the case of a patient with hemianomalous pulmonary venous drainage and intact atrial septum who underwent surgical repair using a pericardial baffle and creation of an “atrial septal defect” aged 15 years. Dyspnoea and recurrent chest infections started 7 months after surgery when he was seen by a respiratory physician without cardiac followup. He presented again aged 28 years with a recurrent pneumonia investigated over 6 weeks and heart pronounced normal from examination and echocardiography. Correct diagnosis was made in Grown Up Congenital Heart (GUCH) clinic stimulating review of data and catheterisation with pulmonary artery angiography which confirmed it. We feel that this case highlights the importance of specialist care and followup for GUCH patients.

Cardiac surgery in grown-up congenital heart patients. Will the surgical workload increase?

Cardiac surgery in grown-up congenital heart patients. Will the surgical workload increase?

Cardiac surgery in grown-up congenital heart patients. Will the surgical workload increase?

The number of patients with grown-up congenital heart (GUCH) disease is steadily increasing. Although there is agreement that the medical service for GUCH patients should be expanded in coming years, it is still unknown whether this should also include the surgical service. In an attempt to elucidate this we reviewed our population of surgical GUCH patients (n=225) operated in our institution from 1998 to 2005. The patients' charts were reviewed. For details of the procedures, the hospital's internal database (PATS) was used. Patients were stratified according to diagnosis and complexity of the surgical procedures in a simple, moderate and complex category group. The yearly number of operations remained stable in the period. However, whereas the size of the simple complexity group was reduced in the study period, the size of both the moderate and the complex category groups increased progressively. Forty-four percent of the surgical procedures were reoperations. Morbidity and mortality (1.3%) were low. The present study suggests that the future surgical GUCH patients will be increasingly complex. However, it is speculative whether the total number of surgical GUCH patients will increase. This is especially explained by the continuous introduction of new percutaneous catheter techniques.

Cardiac transplantation in adults with congenital heart disease

Objectives: Advances in surgical and medical management of congenital heart disease result in survival of an increasing number of grown-up congenital heart (GUCH) patients. As myocardial dysfunction can occur, some of them require heart transplantation (HTx). Methods and results: Fourteen GUCH patients (mean age 30.6, range 16–58 years) underwent HTx between April 1986 and June 2005 at our institution. The diagnoses were: single ventricle (n=4), d-TGA and previous atrial switch operation (n=2), l-TGA (n=1), Morbus Uhl (n=3), others with VSD and complex valve surgery. Refractory heart failure (NYHA IV) was present in all. Four patients received heart transplants after mechanical circulatory support (3 ECMO, 1 BVAD Berlin Heart). Nine patients had a mean of 2 previous cardiac operations. There was 1 early death due to infection. The Kaplan-Meier survival rate was 93% at 1 year, 84% at 5 and 84% at 10 years. No anatomic or surgical risk factor was predictive of death. Outcome was compared with matched (age, gender, time of HTx) controls consisting of 70 patients with dilated cardiomyopathy (DCM) and 28 with ischemic heart disease (IHD). Survival rates of the control groups did not differ significantly from those of GUCH (1 year, 5 years, 10 years: DCM 92%, 88%, 84%; IHD 9%, 89%, 75%). Conclusions: Successful HTx can be achieved in patients with GUCH disease, with an outcome similar to that of patients with acquired heart disease. Detailed assessment of cardiac anatomy and careful surgical planning are essential to obtain optimal results.

Bridging the gaps: transition for young people with cancer

Gaps are an inevitable outcome of the organisation of patient care around age bands, specialities and diseases. One such gap, that between child and adolescent health services and adult medical care, is particularly problematic for those whose medical problems begin in childhood and continue into adult life [ 1 Viner R.M. Transition from paediatric to adult care. Bridging the gaps or passing the buck?. Arch. Dis. Child. 1999; 81: 271-275 Crossref PubMed Scopus (268) Google Scholar ]. These ‘transition’ issues are beginning to be addressed in classic chronic illnesses, such as asthma [ 2 Sawyer S. Blair S. Bowes G. Chronic illness in adolescents transfer or transition to adult services?. J. Paediatr. Child Health. 1997; 33: 88-90 Crossref PubMed Scopus (119) Google Scholar ] and diabetes [ 3 Sawyer S. The process of transition to adult health care services. in: Werther G. Court J. Diabetes and the Adolescent. Blackwell, Melbourne1997 Google Scholar ]. They have additionally been given particular emphasis by the survival into adult life of cohorts with conditions once considered confined to childhood, such as cystic fibrosis, congenital heart disease [ 4 Sommerville J. Near misses and disasters in the treatment of grown-up congenital heart patients. J. R. Soc. of Med. 1997; 90: 124-127 PubMed Google Scholar ] and metabolic conditions. The appropriate management of transition is now recognised as part of high quality care of adolescents with chronic conditions [ 4 Sommerville J. Near misses and disasters in the treatment of grown-up congenital heart patients. J. R. Soc. of Med. 1997; 90: 124-127 PubMed Google Scholar ].

Atrial and ventricular electromechanical function in 1-ventricle hearts: Influence of atrial flutter and Fontan procedure

Objective: Echocardiography was used to study electromechanical atrial and ventricular function in adult patients with a 1-ventricle heart who were in sinus rhythm to better understand the recurrence of atrial flutter in these conditions. Patients who had recent atrial flutter, with and without the Fontan procedure, were compared with those who had no arrhythmia. Methods: This was a prospective study that used M-mode and 2-dimensional Doppler echocardiography and electrocardiography. Conventional measurements were used to evaluate ventricular long-axis function. Basic data were drawn from case notes. The setting was a designated quaternary service for grown-up congenital heart patients (GUCH) in a tertiary referral center for cardiology and cardiac surgery. From January 1997 to February 1998, 26 consecutive adult patients (aged >16 years) with a heart with one functioning ventricle and a history of atrial flutter were studied: group 1, with non-Fontan palliative surgery or no surgery (10 patients), and group 2, with Fontan-type repair (16 patients). Also studied were 20 patients with a 1-ventricle heart but no history of atrial flutter. These 20 patients were divided into 2 groups: control 1, which comprised 14 patients with previous shunts or no surgery, and control 2, which consisted of 6 patients with Fontan repair. Results: P-wave duration on the electrocardiogram was similar in the 4 patient groups, but the amplitude was reduced in group 2 and control 2 (patients with Fontan surgery) (P <.016). Bifid P wave was seen in 5 (50%) of 10 patients in group 1 and in 6 (43%) of 14 patients in control 1, but it was not seen in patients with Fontan (P <.01). Ventricular systolic and diastolic dimensions and fractional shortening were not different between patients and controls. Right atrial transverse dimensions were greater in group 2 patients compared with those in controls. Significant atrioventricular valve regurgitation was seen in 9 of 10 group 1 patients but in none of the others. Right-sided total long-axis excursion and atrial A-wave amplitude were depressed in group 2 patients compared with the values in the others. The onset of right atrial shortening was delayed by 50 ms in group 2 compared with control 2, whereas the left atrial shortening was delayed by 30 ms in group 1 compared with control 1. This particular disturbance remained 6 months after cardioversion. Conclusion: In 1-ventricle hearts, significant atrioventricular valve regurgitation is commonly associated with atrial flutter in patients who did not undergo the Fontan procedure, and with electromechanical disturbances in those who did. Recognition of disturbances in ventricular long-axis function may thus assist in the identification of patients with a 1-ventricle heart who are prone to atrial flutter. (J Am Soc Echocardiogr 2001;14:186-93.)

Grown-up congenital heart disease--medical demands look back, look forward 2000.

A new medical community, the grown-up congenital heart patients--GUCH--has resulted from successes of cardiac surgery over 30-40 years. Many survivors have complicated problems, medical and surgical, demanding experience and expertise neither provided nor organised in most countries. Islands of good care exist with difficulty. The experience of one specialist GUCH unit established for 25 years shows that 55-60% admissions are for complex lesions, particularly after complicated surgery. The patients' overall costs are at least twice those of other cardiac patients. GUCH admissions are about 5-8% of the total, varying according to the population/region served. Supervised medical care for GUCH is equally important in outpatient services, involving 3 times the secretarial time of other cardiac patients, an accessible database and a "helpline" for doctors and patients. This may be life-saving in patients with complex conditions. The GUCH population is ageing, with increasing numbers of complex patients. 30% of admissions now are over 40 years old, and 5% are over 60, confirming that this is an adult medical speciality, not paediatric. Invasive investigations and arrhythmias provide the most frequent reasons for admissions--atrial flutter is the commonest arrhythmia, needing experts when it occurs in Fontan, transposition, etc. Routine coronary arteriography is also important. In cardiac surgery, one in five admissions presents organisational problems. Reoperation, now as many as 9 or 10 times, has to be optimised. Reoperation on left and right outflow tracts-for changing valves and conduits--is more common than first operations. GUCH patients represent a relatively small portion of the whole population. Such patients in a population of 7-8 million need to be concentrated in 1-2 centres, depending on culture, religion, geography, language etc., to provide necessary experience, expertise and education.

Atrial flutter in grown-up congenital heart (GUCH) patients: Clinical characteristics of affected population

Objective: To define occurrence, lesions and clinical characteristics of grown-up congenital heart (GUCH) patients who develop atrial flutter (AFL). Design: All GUCH patients who presented as inpatients or outpatients with documented sustained AFL between 1996 and 1998 were studied prospectively. Retrospective review of case notes for basic data relating to underlying anomaly, prior surgery and age at onset of AFL, enquiry into events before the first attack. Clinical state was assessed by Ability Index before AFL and at last visit. Setting: Designated quaternary service for GUC in a tertiary referral centre. Results: From October 1996 to April 1998, 100 consecutive patients (49 female) aged 17–77 (mean 35) years, who presented to the GUCH Unit at Royal Brompton Hospital with a sustained attack of AEL documented by a 12 lead electrocardiogram were studied. Four basic cardiac anomalies accounted for 75% patients: one ventricle (26), atrial septal defect (ASD) (19), transposition of great arteries (TGA) (17) and Tetralogy of Fallot (13). AFL occurred occasionally in small ventricular septal defect (VSD), congenital corrected TGA (CC-TGA), pulmonary stenosis and pulmonary atresia with or without VSD. 86/100 patients had undergone cardiac surgery: Fontan 19 (22%), reconstruction of right ventricular outflow tract 17 (20%), closure of ASD 15 (17%), Mustard for TGA 13 (15%), and other palliative surgery 22 (26%). AFL occurred in ‘natural history’ (unoperated) in 14 (14%) mostly in CC-TGA, ASD and Fallot. Age at first attack was 6–64 (mean 28) years with the first attack occurring at younger age after Mustard (22±7 years) and Fontan (24±7), than in un-operated ASD (46±13) and CCTGA (31±10). Haemodynamic abnormalities from anatomical causes were present in 62/74 (84%) patients who had undergone reparative surgery and included venous pathway obstruction, pulmonary regurgitation and pulmonary hypertension. Additional factors which could have precipitated AFL in prone patients were present in 63. New symptoms appeared in 96 patients with the first attack of AFL. Ability Index prior to onset in 90 patients who have been followed-up for more than 1 year since the first onset was 1 in 52, 2 in 31, 3 in 6 and 4 in 1 patients. At the last visit (mean time from the first onset 6.6±4.7 years), only 9 patients remained with Ability Index 1, 43 in 2, 20 in 3 and 18 in 4 despite return to sinus rhythm. Conclusion: One ventricle heart, ASD, transposition of great arteries and Tetrology of Fallot are the most common underlying anomalies in GUCH patients who develop AFL. It is less commonly seen in unoperated patients. When occurs AFL compromises patients’ activities and deteriorates the clinical condition. Residual or developed haemodynamic abnormalities and precipitating factor are often present in this patients, hence full investigation and close follow up are necessary once AFL develops.

Infective endocarditis in the grown-up congenital heart (GUCH) population.

Infective endocarditis accounts for 4% of admissions to a specialized unit for grown-up congenital heart patients. This study defines lesions susceptible to infection, antecedent events, organisms, outcome and surgical treatment in a group of such patients. The grown-up congenital heart disease database was searched for all patients aged 13 years and above with adequate documentation of infective endocarditis retrospectively between 1983-1993 and thereafter between 1993-1996. There were 185 patients (214 episodes) divided into Group I: 128 patients unoperated or palliated and Group II: 57 patients after definitive repair and/or valve repair/replacement. In Group I, the commonest affected sites were ventricular septal defect in 31 (24%), left ventricular outflow tract in 22 (17%) and mitral valve in 17 (13%) and in Group II, left ventricular outflow tract in 20 (35%), repaired Fallot in 11 (19%), and atrioventricular defects in eight (14%). Infective endocarditis was not seen in secundum atrial septal defects before or after closure; in closed ventricular septal defects and ducts without left-sided valve abnormality; in isolated pulmonary stenosis; in unrepaired Ebstein: or after Fontan-type or Mustard operations. Surgery was performed in 39 patients: as an emergency in 17, and for failed medical therapy in 22. Only 87 (41%) of patients had a predisposing event: dental procedure or sepsis were the commonest events in Group I (33%) and cardiac surgery in Group II (50%). Streptococci species were found in 54% of Group I patients and in 45% of Group II. Staphylococci aureus was commoner in Group II (25%) compared to Group I (14%). Mean time from the onset of symptoms to diagnosis was 60 and 29 days in Groups I and II, respectively. Eight (4%) patients died as a result of septicaemia related to emergency or repeated surgery and Staphylococcus aureus infection. Recurrent attacks occurred in 21 (11%) patients. Reparative surgery does not prevent endocarditis except for closure of a ventricular septal defect and duct. Delay in diagnosis is serious since it contributes to mortality, although the overall mortality % is not high. Specific lesions are not affected so prophylaxis is probably unnecessary in those anomalies.

Cardiac Surgery for Grown-Up Congenital Heart Patients: Survey of 307 Consecutive Operations from 1991 to 1994

The cardiac surgery performed from 1991 to 1994 in a unit dedicated specifically for grown-up congenital heart (GUCH) patients was reviewed to determine the frequency of various procedures, incidence of first and reoperations, early mortality, and its determinants. The 295 patients, aged 16 to 77 years (31 ± 13), had 307 operations. First operations (n = 128, 42%) were most commonly for closure of atrial septal defect (n = 40), aortic valve replacement (n = 31) or repair of aortic coarctation (n = 14). Reoperations were more frequent (n = 179, 58%) and divided among first corrective repair (n = 49), reoperation after corrective repair (n = 115), and further palliation (n = 15). First corrective surgery was mainly for aortic valve disease (n = 17), Fallot (n = 7), and lesions needing a Fontan procedure (n = 5). Reoperations after corrective repair were needed for aortic valve disease (n = 43), right-sided conduit (n = 30), or recoarctation (n = 11). Early mortality was influenced by presence of central cyanosis (9 of 49, 18% in cyanotic patients; 12 of 258, 5% in acyanotic; p <0.001), increased number of previous operations (0 = 4%, 1 = 7%, 2 = 11%, >2 = 13%; p = 0.003), and increasing age of patients. Cyanotic patients had more serious postoperative complications: pleural and pericardial effusions, severe bleeding, renal insufficiency, and sepsis, and their hospital stay was longer compared with acyanotic patients (20 ± 17 vs 11 ± 8 days; p <0.001). In GUCH patients, reoperations cause the largest demand on cardiac surgical services. Increased survival of patients with complex cardiovascular malformations brings difficult challenges not only to cardiologists but also to cardiovascular surgeons. There is a need to provide continued highly specialized care. Resources, patients, and funding should be concentrated in a few designated centers.