Reintervention and survival in 1428 patients in the Australian and New Zealand Fontan Registry.

OBJECTIVE: Patients undergoing single-ventricle palliation have experienced significant improvement in survival in the recent era. However, a substantial proportion of these patients undergo reoperations. We performed a review of the Australia and New Zealand (ANZ) Fontan Registry to determine the overall reintervention and reoperative burden in these patients. METHODS: A retrospective longitudinal cohort study was performed using data from patients who underwent a Fontan operation between 1975 and 2016 from the ANZ Fontan Registry. The data obtained included Fontan operation, reinterventions and most recent follow-up status. We examined the type and timing of reinterventions and survival. RESULTS: Of the 1428 patients identified, 435 (30%) underwent at least one reintervention after the Fontan operation: 110 patients underwent early reintervention and 413 underwent late reinterventions. Excluding Fontan conversion and transplantation, 220 patients underwent at least one interventional procedure and 209 patients underwent at least one reoperation. Fenestration closure and pacemaker-related procedures were the most common catheter and surgical interventions, respectively. The cumulative incidence of reintervention following Fontan was 23%, 37% and 55% at 10, 20 and 30 years, respectively. Survival and freedom from failure were worse in patients requiring later reintervention after Fontan surgery (51% vs 83% and 42% vs 69%, respectively at 30 years, p<0.001). This difference persisted after excluding pacemaker-related procedures (p<0.001). Operative mortality for non-pacemaker late reoperations after Fontan was 6%. CONCLUSIONS: A substantial proportion of Fontan patients require further intervention to maintain effective single-ventricle circulation. Patients undergoing reoperation after Fontan have higher rates of mortality and failure, despite intervention.

Pacemakers are associated with a higher risk of late death and transplantation in the Fontan population.

BACKGROUND: The need for permanent pacing has been identified as a predictor of poor outcomes in the late survivors of Fontan surgery. However, it is not clear if the need for a pacemaker is a surrogate marker of a declining Fontan state, or if pacing is deleterious to the Fontan circulation. OBJECTIVES: We sought to compare the long-term outcomes of propensity-matched Fontan patients with and without a permanent pacemaker. METHODS: Patients who have survived Fontan completion with a documented history of cardiac arrhythmia were identified from the Australia and New Zealand Fontan Registry. Pacemaker insertion details, cardiac function and electrophysiological data were obtained for the patients with a permanent pacemaker. Survival analysis was performed with propensity score matching to compare late survival and outcomes in patients with versus without a pacemaker. RESULTS: There was a total of 310 patients with a history of cardiac arrhythmia, of which 126 (41%) had a permanent pacemaker. After propensity-score matching, 99 pairs were generated (n = 198). Patients with a permanent pacemaker had a higher risk of death (HR 3.32 95% CI 1.60-6.90, p = 0.001) and death or transplantation (HR 3.55 95% CI 1.87-6.73, p < 0.001). Patients who were only paced atrially were not at a significantly increased risk of death or transplantation. However, patients who were ventricular paced >50% of the time were much more likely to encounter late death or transplantation (HR 3.82 95% CI 1.64-8.95, p = 0.002). CONCLUSIONS: Having a permanent pacemaker and needing ventricular pacing is likely associated with an increased risk of death and transplantation in patients with a Fontan circulation.

Hepatic and renal end-organ damage in the Fontan circulation: A report from the Australian and New Zealand Fontan Registry.

BACKGROUND: Hepatic and renal dysfunction have been observed in survivors of the Fontan procedure, however their incidence and associated factors remain poorly defined. METHODS: A total of 152 participants from a Registry of 1528 patients underwent abdominal ultrasound, transient elastography (FibroScan), serum fibrosis score (FibroTest), in vivo Tc-99m DTPA measurement of glomerular filtration rate (mGFR), and urine albumin-creatinine ratio (ACR). RESULTS: Mean age and time since Fontan were 19.8 ±â€¯9.3 and 14.1 ±â€¯7.6 years, respectively. Features suggestive of hepatic fibrosis were observed on ultrasound in 87/143 (61%) and no patient was diagnosed with hepatocellular carcinoma. FibroScan median kPa was ≥10 in 117/133 (88%), ≥15 in 75/133 (56%), and ≥20 in 41/133 (31%). Fifty-four patients (54/118, 46%) had a FibroTest score ≥0.49 (equivalent to ≥F2 fibrosis). FibroTest score correlated with FibroScan value (r = 0.24, p = 0.015) and ACR (r = 0.29, p = 0.002), and patients with ultrasound features of hepatic fibrosis had a higher FibroScan median kPa (19.5 vs 15.4, p = 0.002). Renal impairment was mild (mGFR 60-89 ml/min/1.73 m2) in 46/131 (35%) and moderate (mGFR 30-59 ml/min/1.73 m2) in 3/131 (2%). Microalbuminuria was detected in 52/139 participants (37%). By multivariable analysis, time since Fontan was associated with increased FibroScan median kPa (ß = 0.89, 95% CI 0.54-1.25, p = 0.002) and decreased mGFR (ß = -0.77, 95% CI -1.29-0.24, p = 0.005). CONCLUSIONS: In the second decade after Fontan hepatic and renal structure and function are abnormal in a significant number of patients: close to 60% have ultrasonographic evidence of structural hepatic abnormalities, 46% have elevated serum hepatic fibrosis scores, and 57% have either reduced glomerular filtration rate or microalbuminuria. Hepatic and renal function should be monitored for potential impacts on outcomes after Fontan completion.

Long-term performance of homografts versus stented bioprosthetic valves in the pulmonary position in patients aged 10-20 years.

OBJECTIVES: We aimed to compare the long-term performance of pulmonary homografts and stented bioprosthetic valves in the pulmonary position in patients aged 10-20 years. METHODS: Between January 1995 and December 2015, 188 patients aged 10-20 years undergoing pulmonary valve replacement were identified retrospectively from hospital databases in both congenital cardiac centres in Brisbane. Valve performance was evaluated using previously described standard criteria. Propensity score matching was used to balance the 2 treatment groups. RESULTS: Freedom from structural valve degeneration in homografts (n = 131) was 97%, 92% and 85% at 3, 5 and 10 years, respectively, and 91% and 53% at 3 and 5 years, respectively, in the bioprosthesis group (n = 57). Freedom from reintervention in homografts was 96%, 93% and 88% at 3, 5 and 10 years, respectively, and 93% and 68% at 3 and 5 years, respectively, in the bioprosthesis group. The unadjusted Cox regression analysis demonstrated that a bioprosthesis was at 5.64 times the risk of structural valve degeneration and 3.89 times the risk of reintervention. The Cox regression analysis performed on the propensity matched sample (45 pairs of patients) revealed that a bioprosthesis was at almost 10 times the risk of experiencing structural valve degeneration [hazard ratio (HR) = 9.18] and at more than 8 times the risk of undergoing a reintervention (HR = 8.34). CONCLUSIONS: In our patient population, pulmonary homografts outperformed stented bioprosthetic valves within 5 years when implanted in the pulmonary position in patients aged 10-20 years. We recommend the use of a pulmonary homograft for pulmonary valve replacement in this age group in patients undergoing surgery for congenital heart disease.

Long-term outcomes of single-ventricle palliation for pulmonary atresia with intact ventricular septum: Fontan survivors remain at risk of late myocardial ischaemia and death.

OBJECTIVES: The specific outcomes of patients with pulmonary atresia with intact ventricular septum late after Fontan palliation are unknown. Patients with smaller right ventricles and myocardial sinusoids are known to have worse survival in the first years of life. Whether the potential for coronary ischaemia affects the long-term outcomes of these patients after Fontan palliation is still unknown. METHODS: All patients with pulmonary atresia with intact ventricular septum who underwent the Fontan procedure from 1984 to 2016 in Australia and New Zealand were identified, and preoperative, perioperative and follow-up data were collected. RESULTS: Late follow-up data were available for 120 patients. The median length of follow-up after the Fontan procedure was 9.1 years (interquartile range 4.2-15.4 years). Late death occurred in 9% of patients (11/120). Six were sudden, unexpected deaths; 4 of those occurred in patients known to have right ventricle-dependent coronary circulation (RVDCC). Those with RVDCC had a higher incidence of sudden death (4/20 vs 2/100; P = 0.007). RVDCC was associated with late death (P = 0.01) and the development of myocardial ischaemia after Fontan completion (P < 0.001). The 10-year survival rate was 77% (95% confidence interval 56-100%) for patients with RVDCC vs 96% (95% confidence interval 92-100%) for patients without RVDCC. CONCLUSIONS: Long-term survival of patients with pulmonary atresia with intact ventricular septum after the Fontan procedure is excellent, but patients with RVDCC remain susceptible to coronary ischaemia and sudden death. Closer surveillance and investigation for exercise-induced ischaemia may be necessary.

Propensity score matched analysis of partial atrioventricular septal defect repair in infancy.

OBJECTIVE: Partial atrioventricular septal defect (pAVSD) is usually repaired between 2 and 4 years of age with excellent results. Repair during infancy has been associated with poorer outcomes. However, most infants in reported series had heart failure or significant left atrioventricular valve (LAVV) regurgitation. The impact of surgery during infancy on outcomes remains unclear. METHODS: All children at three institutions who underwent repair of pAVSD from 1975 to 2015 were included. Infants (aged <1 year) were compared with older children in a propensity score matched analysis. Variables used to generate propensity scores were: failure to thrive, congestive heart failure, preoperative LAVV regurgitation, associated congenital heart disease, sex and the presence of trisomy 21. RESULTS: pAVSD repair was performed on 430 children, 17.4% (75/430) were infants. Infants (mean age 0.5±0.3 years) had higher rates of LAVV regurgitation, heart failure and additional cardiac malformations than older children (mean age 4.7±3.5 years). At 30 years, survival for infants was 82.1% (95% CI 70.1% to 89.6%) compared with 95.7% (95% CI 91.3% to 97.9%) in older children (P<0.001).Propensity score matching yielded 52 well-matched pairs. Survival at 30 years was 87.9% (95% CI 75.0% to 94.4%) for infants compared with 98.1% (95% CI 87.1% to 99.7%) for older children (P=0.04). There was no significant difference in freedom from reoperation between the groups. CONCLUSIONS: Despite matching for risk factors, survival after repair of pAVSD during infancy is lower than that when repair is performed in older children, with no difference in reoperation rates. This suggests that elective repair of pAVSD should be deferred until after infancy.

Twenty-Five Year Outcomes of the Lateral Tunnel Fontan Procedure.

The objective of this study was to characterize late outcomes of the lateral tunnel (LT) Fontan procedure. The outcomes of all patients who underwent an LT Fontan procedure in Australia and in New Zealand were analyzed. Original files were reviewed and outcomes data were obtained through a binational registry. Between 1980 and 2014, a total of 301 patients underwent an LT Fontan procedure across 6 major centers. There were 13 hospital mortalities, 21 late deaths, 8 Fontan conversions and revisions, 8 Fontan takedowns, and 4 heart transplantations. Overall survival at 15 and 25 years was 90% (95% confidence interval [CI]: 86%-93%) and 80% (95% CI: 69%-91%), respectively. Protein-losing enteropathy or plastic bronchitis was observed in 14 patients (5%). Freedom from late failure at 15 and 25 years was 88% (95% CI: 84%-92%) and 82% (95% CI: 76%-87%), respectively. Independent predictors of late Fontan failure were prolonged pleural effusions post Fontan operations (hazard ratio [HR] 3.06, 1.05-8.95, P = 0.041), age >7 years at Fontan (vs 3-5 years, HR 9.7, 2.46-38.21, P = 0.001) and development of supraventricular tachycardia (HR 4.67, 2.07-10.58, P < 0.001). Freedom from tachy- or bradyarrhythmias at 10 and 20 years was 87% (95% CI: 83%-91%) and 72% (95% CI: 66%-79%), respectively. Thromboembolic events occurred in 45 patients (16%, 26 strokes), and freedom from symptomatic thromboembolism at 10 and 20 years was 93% (95% CI: 89%-96%) and 80% (95% CI: 74%-86%), respectively. Over a 25-year period, the LT technique has achieved excellent late survival. As this population ages, it is at an increasing risk of failure and adverse events. We are likely to see an increasing proportion requiring heart transplantation and late reintervention.

Hospital Inpatient Costs for Single Ventricle Patients Surviving the Fontan Procedure.

We estimated the inpatient resource use for a Fontan patient from birth to adulthood and explored factors that might induce cost differences (2014 US dollar). Inpatient costing records from 4 hospitals with greatest numbers of Fontan patients in Australia and New Zealand were linked with the Fontan registry database. Inpatient records between July 1995 and September 2014 for 420 Fontan patients were linked, and the most frequent primary diagnoses were hypoplastic left heart syndrome (20.7%), tricuspid atresia (19.7%), and double inlet left ventricle (17.1%). The mean hospital cost for a Fontan patient from birth to 18 years of age was estimated to be $390,601 (95% confidence interval [CI] $264,703 to $516,499), corresponding to 164 (95% CI 98 to 231) inpatient days. The cost incurred from birth through to Fontan completion (the staged procedures period) was $219,482 (95% CI $202,410 to $236,553) and the cost thereafter over 15 years was $146,820 (95% CI $44,409 to $249,231), corresponding to 82 (95% CI 72 to 92) and 65 (95% CI 18 to 112) inpatient days, respectively. Costs were higher in male and hypoplastic left heart syndrome patients in the staged procedures period (p <0.001). Having fenestration was associated with higher costs in the staged procedures period (p <0.001) and lower cost after Fontan over 15 years (p = 0.66). In conclusion, patients with single ventricle congenital heart disease continue to demand considerable inpatient resources after the staged procedures period. Over 40% of the pediatric hospital costs for Fontan patients were estimated to occur after the last planned surgery.

Common atrioventricular valve failure during single ventricle palliation†.

OBJECTIVES: To determine the risk of atrioventricular valve failure (valve intervention or moderate or greater regurgitation) during the lifetime of patients with single ventricle physiology and common atrioventricular valve. METHODS: Patients' data were extracted from an existing bi-national, population based registry. A retrospective review of their medical records was undertaken to determine the incidence of atrioventricular valve repair/replacement or moderate or greater regurgitation. RESULTS: From a registry of 1468 Fontan survivors, 136 patients with common atrioventricular valve were identified. Complete echocardiographic follow-up was available for 114 patients. Median length of follow-up was 10.2 years (interquartile range 5-15 years). Twenty-five year survival and freedom from Fontan failure were 94% [95% confidence interval (CI), 88-100%] and 74% (95% CI, 64-87%), respectively. Twenty-eight patients underwent 24 initial repairs and 4 replacements. The 24 patients undergoing repair subsequently needed 6 re-repairs, 2 replacements and 8 had moderate or greater regurgitation at last follow-up. Four-year freedom from atrioventricular valve repair failure was 50% (95% CI, 34-75%). An additional 30 patients developed moderate or greater atrioventricular valve regurgitation (6 New York Heart Association ≥3, 10 Fontan failures, 0 deaths). Cumulative incidence of the composite endpoint of atrioventricular valve failure at 28 years was 62% (95% CI, 49-74%). CONCLUSIONS: Patients with single ventricle physiology and common atrioventricular valve experience a continuous decline in valve function. The majority of patients experience valve failure in the first 30 years of life.

Long-term outcomes after first-onset arrhythmia in Fontan physiology.

OBJECTIVES: Patients living with a Fontan circulation are prone to develop arrhythmias. However, their prognostic impact has been seldom studied. As such, we aimed to determine the incidence and predictors of arrhythmias after the Fontan procedure and the long-term outcomes after the first onset of arrhythmias. METHODS: Of the 1034 patients who have undergone a Fontan procedure as recorded in the Australian and New Zealand Fontan Registry, we identified those in whom a tachyarrhythmia or bradyarrhythmia developed. We evaluated the incidence and predictors of developing arrhythmias and their prognostic impact on late outcomes. RESULTS: Arrhythmia developed in 195 patients. Tachyarrhythmia was present in 162 patients, bradyarrhythmia was present in 74 patients, and both forms were present in 41 patients. At 20 years, freedom from any arrhythmia, tachyarrhythmia, and bradyarrhythmia was 66% (95% confidence interval [CI], 59-72), 69% (95% CI, 62-75), and 85% (95% CI, 80-90), respectively. On multivariable analyses, patients with an extracardiac Fontan (hazard ratio [HR], 0.23; 95% CI, 0.10-0.51; P < .001) were less likely to develop an arrhythmia, whereas those with left atrial (HR, 3.18; 95% CI, 1.45-6.95; P = .004) and right atrial (HR, 4.00; 95% CI, 2.41-6.61; P < .001) isomerism were more likely to have an arrhythmia. After onset of any arrhythmia (tachyarrhythmia or bradyarrhythmia), 10- and 15-year survivals were 74% (65%-83%) and 70% (60%-80%), respectively, and freedom from Fontan failure was 55% (44%-64%) and 44% (32%-56%), respectively. The development of any arrhythmia (HR, 2.20; 95% CI, 1-44-3.34; P < .001), tachyarrhythmia (HR, 2.56; 95% CI, 1.60-4.11; P < .001), and bradyarrhythmia (HR, 1.85; 95% CI, 1.16-2.95; P = .01) were all independent predictors of late Fontan failure on multivariable analyses. CONCLUSIONS: The development of an arrhythmia is associated with a heightened risk of subsequent failure of the Fontan circulation.

No difference between aspirin and warfarin after extracardiac Fontan in a propensity score analysis of 475 patients.

OBJECTIVES: The most effective method of long-term thromboprophylaxis after the Fontan procedure is not clear. We compared the rates of thromboembolic events between patients receiving aspirin and warfarin after an extracardiac conduit Fontan procedure in a bi-national registry. METHODS: All patients who underwent an extracardiac conduit Fontan procedure from 1997 to 2010 in Australia and New Zealand were identified. Medication status and routine follow-up and echocardiographic data were obtained from all patients. Survival analysis with propensity score matching and adjustment was performed to determine the treatment effect of warfarin compared with that of aspirin beyond the first year of follow-up, after which time patients had settled on their long-term thromboprophylaxis strategy. RESULTS: Of 570 eligible patients, the data of 475 patients who were regularly followed up without mechanical valve replacement were available for analysis. Long-term thromboprophylaxis consisted of warfarin in 301 patients (63%), aspirin in 157 (33%) and none in 17 (4%). The 10-year rate of freedom from all thromboembolic events was 91% [95% confidence interval (CI) 88-94%]. Thromboembolic events beyond the first year of follow-up occurred in 18 patients (6 on aspirin and 12 on warfarin). After (i) propensity score adjustment and (ii) matching yielding 164 pairs, the hazard rates of thromboembolic events beyond the first year were not statistically different between the warfarin and aspirin groups [(i) hazard ratio (HR) 2.3, 95% CI 0.7-7.4, P = 0.2 and (ii) HR 1.5, 95% CI 0.5-4.7, P = 0.5, respectively]. CONCLUSIONS: No difference in the hazard rates of late thromboembolic events was observed between aspirin and warfarin beyond the first year after the extracardiac conduit Fontan procedure.

Outcomes of Truncus Arteriosus Repair in Children: 35 Years of Experience From a Single Institution.

We evaluated the long-term outcomes following repair of truncus arteriosus (TA) from a single institution. We conducted a retrospective review of children (n = 171) who underwent TA repair between 1979 and 2014. Early mortality rate was 11.7% (20/171). There were 19 late deaths. Most deaths (74%, 29/39) occurred within the first year following surgery. The 1-year mortality rate in 1979-2004 was 18% (25/136) and decreased to 11% (4/35) in 2005-2014. The overall survival rate was 73.6% at 30 years. Multivariate analysis identified postoperative extracorporeal membrane oxygenation (P = 0.003), operative weight <2.5kg (P = 0.012), prior surgical intervention (P = 0.018), and coronary artery anomaly (P = 0.037) as risk factors for early mortality. A Cox regression model identified DiGeorge syndrome (P = 0.008) as a risk factor for late mortality. Freedom from right ventricular outflow tract reoperation was 4.6% at 20 years. Concomitant truncal valve (TV) repair or replacement was undertaken in 20 patients. Additionally, 14 patients underwent late TV repair or replacement. The overall survival rate in patients who underwent TV operation was 76.9% at 20 years. A total of 19 patients had concomitant interrupted aortic arch with a survival rate of 89.5% at 20 years. Median follow-up was 19 years (mean = 17 years, range: 1-34 years). All patients were in New York Heart Association Class I/II at last follow-up. Following repair of TA, patients had good long-term functional status but had high reoperation rates. Repair of interrupted aortic arch and TV were not risk factors for mortality. Postoperative extracorporeal membrane oxygenation, operative weight <2.5kg, prior surgical intervention, and coronary artery anomaly were risk factors for early death. DiGeorge syndrome was associated with late death, most commonly from infection.

The extracardiac conduit Fontan procedure in Australia and New Zealand: hypoplastic left heart syndrome predicts worse early and late outcomes.

OBJECTIVES: To identify factors associated with hospital and long-term outcomes in a binational cohort of extracardiac conduit (ECC) Fontan recipients. METHODS: All patients who underwent an ECC Fontan procedure from 1997 to 2010 in Australia and New Zealand were identified, and perioperative, follow-up, echocardiographic and reintervention data collected. Risk factors for early and late mortality, failure and adverse outcomes were analysed. RESULTS: A total of 570 patients were identified, and late follow-up was available in 529 patients. The mean follow-up was 6.7 years (standard deviation: 3.5) and completeness of the follow-up was 98%. There were seven hospital mortalities (1%) and 21 patients (4%) experienced early failure (death, Fontan takedown/revision or mechanical circulatory support). Prolonged length of stay occurred in 10% (57 patients), and prolonged effusions in 9% (51 patients). Overall survival at 14 years was 96% (95% confidence interval [CI]: 93-98%), and late survival for patients discharged with intact Fontan was 98% (95% CI: 94-99%). The rates of late failure (late death, transplantation, takedown, New York Heart Association class III/IV or protein-losing enteropathy) and adverse events (late failure, reoperation, percutaneous intervention, pacemaker, thromboembolic event or supraventricular tachycardia) per 100 patient-years were 0.8 and 3.8, and their 14-year freedoms were 83% (95% CI: 70-91%) and 53% (95% CI: 41-64%), respectively. After adjustment for confounders, hypoplastic left heart syndrome (HLHS) was strongly associated with prolonged effusions (OR: 2.9, 95% CI: 1.4-5.9), late failure (hazard ratio [HR]: 2.8, 95% CI: 1.1-7.5) and adverse events (HR: 3.6, 95% CI: 1.3-7.5). CONCLUSIONS: The extracardiac Fontan procedure provides excellent survival into the second decade of life, but half of patients will suffer a late adverse event by 14 years. Patients with HLHS are at higher risk of late adverse events than other morphological groups, but their survival is still excellent.

The management of the older adult patient with anomalous left coronary artery from the pulmonary artery syndrome: a presentation of two cases and review of the literature.

ALCAPA (anomalous left coronary artery from pulmonary artery) syndrome is a rare congenital abnormality that involves an anomalous insertion of the left coronary artery into the pulmonary artery. Ninety percent of patients present in the first year of life with signs and symptoms of heart failure or sudden cardiac death secondary to chronic myocardial ischemia. There have been an increasing number of reports of ALCAPA patients surviving to adulthood. There seems, however, to be a tendency to die suddenly in the third decade of life. Adult survivors are either asymptomatic or present with mitral regurgitation, cardiomyopathy, myocardial ischemia, or malignant arrhythmias. The management of the older patient presenting with symptoms resulting from ischemia and progressive left ventricular dysfunction remains a challenge. Treatment is largely based on guidelines for adult congenital heart disease management and an extrapolation of evidence from heart failure practice. Currently, surgical reimplantation of the anomalous coronary onto the aorta is the mainstay of treatment. The management of heart failure, sudden cardiac death, and ventricular arrhythmia present problems that are not addressed by reimplantation of the anomalous vessel alone. In this report, we present two cases with different modes of presentation and discuss treatment options.

Long-term complications following surgical patch closure of multiple muscular ventricular septal defects.

BACKGROUND: Multiple muscular ventricular septal defects (VSDs) in children can be difficult to treat and a range of techniques has been advocated. These include pulmonary artery banding, interventional catheter closure, and a variety of surgical approaches. When there are apical muscular defects and associated coarse trabeculations in the right ventricle (RV) producing a "Swiss cheese" pattern, a large patch extending on to the RV free wall and excluding part of the apex has been used. METHODS: We assessed four adult patients who had surgery 22 to 45 years ago to treat muscular VSD by patches which excluded the RV apex. RESULTS: Ages ranged from 22 to 50 years. Re-presentations were for polycythemia, cyanosis, syncope, and atrial flutter. Echocardiography showed bidirectional flow from left ventricle to apex of RV, no pulmonary hypertension, small-sized RV with diastolic dysfunction, enlarged right atria, reopening of patent foramen ovale (PFO) in three, and positive bubble studies with right to left shunting in two. Catheterization confirmed elevated right atrial and RV end diastolic pressures. Two patients had evidence of hepatic cirrhosis. One woman had device closure of PFO, but has right heart failure. One man had redo surgical closure of VSD and PFO. Another patient is being considered for a Glenn shunt to take some load off RV. CONCLUSIONS: Surgical closure of muscular VSD by large patch with RV apical exclusion gives good early results. However, long term in adult life, the reduced size of RV, and diastolic dysfunction cause problems. These include reopening of PFO with cyanosis, right heart failure, cirrhosis, and arrhythmias.

Treatment of haemoptysis in pulmonary atresia with tranexamic acid.

We report the case of a young woman with continuing haemoptysis, pulmonary atresia, previous shunt surgery, and pulmonary hypertension. She was not suitable for further surgery or for therapeutic embolisation of bronchial vessels. Treatment with tranexamic acid resolved the haemoptysis.

Familial muscular ventricular septal defects and aneurysms of the muscular interventricular septum.

We describe 3 siblings with muscular ventricular septal defects, two requiring surgical closure. One of their offspring had a rare congenital aneurysm of the muscular ventricular septum, also requiring surgery. Another had a small muscular ventricular septal defect which closed spontaneously. Their father had echocardiographic evidence suggestive of a closed muscular defect. Paternal cousins have had ventricular septal defect, hypertrophic cardiomyopathy, and tetralogy of Fallot. There was no evidence of 22q11 deletion. Although ventricular septal defects are the most common congenital heart defect, such familial clustering is uncommon. The distribution of cases in this family suggests autosomal dominant inheritance. With echocardiography, and more precise diagnosis of defects which close, a larger genetic component may be revealed in other families.

Pregnancy and the Rastelli operation.

BACKGROUND: The aim of surgical correction of congenital heart disease is for the patient to live a relatively normal life, including potential pregnancy and childbirth. The Rastelli repair operation using a valved conduit for certain complex congenital heart lesions has resulted in survival into adult life. AIMS: We reviewed reproductive outcome of 22 women over 18 years of age who were survivors of the Rastelli operation performed between 1977 and 1999. RESULTS: Six women had a total of 12 pregnancies with six live births. Three women, with complex transposition of the great arteries, developed more severe subaortic obstruction during pregnancy. All required further heart surgery. Delivery was by Caesarean section in three patients and vaginally in the other three. All infants were healthy and free from defects. Two women had tubal ligations because of their cardiac problems. CONCLUSIONS: Women who have had Rastelli repair operation for complex congenital heart disease can have successful pregnancies. Subaortic stenosis can become a problem and should ideally be eliminated before pregnancy. Because of multiple operations, complications and genetic concerns, some women choose not to reproduce. Careful cardiac evaluation before, during and after pregnancy is essential.

Anomalous origin of the left anterior descending artery from the pulmonary artery associated with bicuspid aortic valve and aortic coarctation.

A 15-year-old boy presented with exertional palpitations and chest pain. Investigation revealed anomalous origin of his left anterior descending coronary artery from his pulmonary trunk causing myocardial ischaemia. He previously had aortic coarctation repair with known aortic root dilation and a bicuspid aortic valve. His left anterior descending artery was implanted into the aortic root using a Gortex interposition conduit. This represents an interesting combination of cardiac abnormalities for which repair required consideration of the requirement of further surgery in the future.