Maarten Witsenburg

Seventeen years of adult congenital heart surgery: a single centre experience §

OBJECTIVEnWith a growing number of children with congenital heart disease (CHD) reaching adulthood, an extensive experience with cardiac surgery in adults with CHD is accumulating. To increase insight in this patient category we report our 17-year single centre experience including predictors for adverse outcome and EuroSCORE performance.nnnMETHODSnPatients and operative characteristics of all consecutive adult CHD patients operated upon between January 1990 and January 2007 were collected. Categorisation was done according to the EACTS/STS congenital database. Early and late morbidity and mortality were assessed with follow-up extending up to 17 years. EuroSCORE performance was assessed.nnnRESULTSnNine hundred and sixty-three procedures were performed in 830 patients (mean age 39.3 years, 50.3% male). A total of 49% were re-do procedures, frequent procedures were for left heart lesions (37%), right heart lesions (31%) and septal defects (8%). The 51% primary procedures largely consisted of less complex procedures but also included 1.4% of tetralogy of Fallot repairs, 4.1% of aortic coarctation repairs and 2.7% of Ebsteins disease repairs. Thirty-day mortality was 1.5% (n=14); predicted mortality by logistic EuroSCORE was 4.6%. c-index was 0.61 (95% CI 0.46-0.75). Major complications such as tamponade requiring intervention occurred in 3.2%, postoperative bleeding requiring re-exploration in 7.1% and renal insufficiency requiring dialysis in 4 (0.4%). Pulmonary hypertension was a strong predictor for short-term mortality; impaired ventricular function and cyanosis for long-term mortality. Overall 17-year survival was 71% (95% CI 61%-82%). Eighty percent of patients were in NYHA class I at last follow-up, 17% in II, 3% in III, 0% in IV.nnnCONCLUSIONSnSurgery in adult CHD patients can be performed with low operative mortality and good clinical outcome. EuroSCORE is not a good model for risk assessment in this group of patients.

Electrocardiographic Criteria for Left Ventricular Hypertrophy in Children

Previous studies to determine the sensitivity of the electrocardiogram (ECG) for left ventricular hypertrophy (LVH) in children had their imperfections: they were not done on an unselected hospital population, several criteria used in adults were not applied to children, and obsolete limits of normal for the ECG parameters were used. Furthermore, left ventricular mass (LVM) was taken as the reference standard for LVH, with no regard for other clinical evidence. The study population consisted of 832 children from whom a 12-lead ECG and an M-mode echocardiogram were taken on the same day. The validity of the ECG criteria was judged on the basis of an abnormal LVM index, either alone or in combination with other clinical evidence. The ECG criteria were based on recently established age-dependent normal limits. At 95% specificity, the ECG criteria have low sensitivities (<25%) when an elevated LVM index is taken as the reference for LVH. When clinical evidence is also taken into account, the sensitivity improved considerably (<43%). Sensitivities could be further improved when ECG parameters were combined. The sensitivity of the pediatric ECG in detecting LVH is low but depends strongly on the definition of the reference used for validation.

Right ventricular outflow tract reconstruction with an allograft conduit in patients after tetralogy of fallot correction: Long-term follow-up

BACKGROUNDnIn tetralogy of Fallot (TOF) pulmonary regurgitation is a frequent complication after initial repair. The objective of the present study was to describe the long-term experience with the use of allograft conduits for right ventricular outflow tract (RVOT) reconstruction after correction of TOF in our institution.nnnMETHODSnBetween 1987 and 2009, 133 allografts were implanted in 126 patients (mean age, 27.8 years). The mean time from initial TOF repair to allograft implantation was 20.8±8.8 years. Kaplan-Meier analyses were done for patient survival, freedom from allograft replacement and freedom from any cardiovascular event.nnnRESULTSnHospital mortality was 1.5% (2 patients). Mean follow-up was 8.1 years. Ten other patients died during late follow-up, in 8 patients the cause was heart failure. Patient survival was 95% at 5 years, 91% at 10 years, and 80% at 15 years. Male sex, older patient age at the time of operation, and the use of preoperative diuretics were associated with increased risk of mortality during follow-up. Freedom from allograft replacement was 83% at 10 years and 70% at 15 years. Freedom from any valve-related event was 80% at 10 years and 67% at 15 years.nnnCONCLUSIONSnRight ventricular outflow tract reconstruction after previous TOF repair can be performed with low risk and a low reintervention rate. Allograft conduits function satisfactorily in the pulmonary position at longer-term follow-up. Functional status after allograft implantation in patients with a previous correction of TOF remains good. There is concern about the long-term survival and the occurrence of heart failure.

Long term follow up after surgery in congenitally corrected transposition of the great arteries with a right ventricle in the systemic circulation

Aim of the studyTo investigate the long-term outcome of surgical treatment for congenitally corrected transposition of the great arteries (CCTGA), in patients with biventricular repair with the right ventricle as systemic ventricle.MethodsA total of 32 patients with CCTGA were operated between January 1972 and October 2008. These operations comprised 18 patients with a repair with a normal left ventricular outflow tract, 11 patients with a Rastelli repair of the left ventricle to the pulmonary artery and 3 patients with a cardiac transplantation.ResultsExcluding the cardiac transplantation patients, mean age at operation was 16 years (sd 15 years, range 1 week - 49 years). Median follow-up was 12 years (sd 10 years, range 7 days - 32 years). Survival obtained from Kaplan-Meier analysis at 20 years after surgery was 63% (CI 53-73%). For the non-Rastelli group these data at 20 years were 62% (CI 48-76%) and for the Rastelli group 67% (CI 51-83%). Freedom of reoperation at 20 years was 32% (CI 19-45%) in the overall group. In the non-Rastelli group the data at 20 years were 47% (CI 11-83%) and for the Rastelli group 21% (CI 0-54%) after almost 19 years.ConclusionsLong term follow up confirms that surgery in CCTGA with the right ventricle as systemic ventricle has a suboptimal survival and limited freedom of reoperation. Death occurred mostly as a result of cardiac failure.

Clinical outcome and health-related quality of life after right-ventricular-outflow-tract reconstruction with an allograft conduit

OBJECTIVEnAllograft conduits are used for reconstruction of the right ventricular outflow tract in congenital heart malformations (biventricular repair) and autograft procedures. A retrospective evaluation of allograft reconstruction of the right-ventricular-outflow-tract reconstruction was conducted and a cross-sectional quality of life study was performed.nnnMETHODSnBetween August 1986 and March 2009, 509 allografts (435 pulmonary and 74 aortic) were implanted in 463 pediatric and adult patients (308 right-sided congenital heart malformations and 155 autograft procedures). Perioperative and follow-up data were collected and analyzed. Kaplan-Meier analyses were done for survival, valve-related re-operation, and valve-related events. Cox regression analysis was used for evaluation of potential risk factors. In addition, the Short Form-36 was presented to patients to assess the perceived quality of life. The results of the Short Form-36 were compared to age-adjusted Dutch population norms.nnnRESULTSnThe mean age at allograft implantation was 19 years (1 week-66 years). Mean follow-up was 9 years (2 days-22 years). Forty-eight patients died during follow-up. Patient survival was 93% at 10 years and 88% at 15 years. A total of 63 re-operations were required for allograft dysfunction in 58 patients. Freedom from valve-related re-operation was 89% at 10 years and 81% at 15 years. Freedom from valve-related events was 86% at 10 years and 74% at 15 years. Younger patient age (p=0.007) and the use of an aortic allograft (p<0.001) were identified as independent risk factors for allograft re-operation. Patients between 14 and 40 years scored significantly lower on physical functioning and general health subscales than the general Dutch population, but scored better on the subscales emotional role functioning and bodily pain. Except for the subscale general health, on which patients within our study population scored lower, patients between 41 and 60 years had comparable average scores as the general Dutch population. The older patient group (61 years or older) had a better average score on the subscale bodily pain and similar scores on other subscales with respect to the general Dutch population.nnnCONCLUSIONSnRight-ventricular-outflow-tract reconstruction with an allograft conduit can be performed with good patient survival, acceptable long-term allograft durability, and good perceived quality of life.

Time Course of Atrial Fibrillation in Patients With Congenital Heart Defects

Background—The incidence of atrial fibrillation (AF) is rising in the aging patients with congenital heart defects (CHD). However, studies reporting on AF in patients with CHD are scarce. The aim of this multicenter study was to examine in a large cohort of patients with a variety of CHD: (1) the age of onset and initial treatment of AF, coexistence of atrial tachyarrhythmia and (2) progression of paroxysmal to (long-standing) persistent/permanent AF during long-term follow-up. Methods and Results—Patients (n=199) with 15 different CHD and documented AF episodes were studied. AF developed at 49±17 years. Regular atrial tachycardia (AT) coexisting with AF occurred in 65 (33%) patients; 65% initially presented with regular AT. At the end of a follow-up period of 5 (0–24) years, the ECG showed AF in 81 patients (41%). In a subgroup of 114 patients, deterioration from paroxysm of AF to (long-standing) persistent/permanent AF was observed in 29 patients (26%) after only 3 (0–18) years of the first AF episode. Cerebrovascular accidents/transient ischemic attacks occurred in 26 patients (13%), although a substantial number (n=16) occurred before the first documented AF episode. Conclusions—Age at development of AF in patients with CHD is relatively young compared with the patients without CHD. Coexistence of episodes of AF and regular AT occurred in a considerable number of patients; most of them initially presented with regular AT. The fast and frequent progression from paroxysmal to (long-standing) persistent or permanent AF episodes justifies close follow-up and early, aggressive therapy of both AT and AF.

Surgical Outcome of Discrete Subaortic Stenosis in Adults A Multicenter Study

Background— Discrete subaortic stenosis is notable for its unpredictable hemodynamic progression in childhood and high reoperation rate; however, data about adulthood are scarce. Methods and Results— Adult patients who previously underwent surgery for discrete subaortic stenosis were included in this retrospective multicenter cohort study. Mixed-effects and joint models were used to assess the postoperative progression of discrete subaortic stenosis and aortic regurgitation, as well as reoperation. A total of 313 patients at 4 centers were included (age at baseline, 20.2 years [25th–75th percentile, 18.4–31.0 years]; 52% male). Median follow-up duration was 12.9 years (25th–75th percentile, 6.2–20.1 years), yielding 5617 patient-years. The peak instantaneous left ventricular outflow tract gradient decreased from 75.7±28.0 mm Hg preoperatively to 15.1±14.1 mm Hg postoperatively (P<0.001) and thereafter increased over time at a rate of 1.31±0.16 mm Hg/y (P=0.001). Mild aortic regurgitation was present in 68% but generally did not progress over time (P=0.76). A preoperative left ventricular outflow tract gradient ≥80 mm Hg was a predictor for progression to moderate aortic regurgitation postoperatively. Eighty patients required at least 1 reoperation (1.8% per patient-year). Predictors for reoperation included female sex (hazard ratio, 1.53; 95% confidence interval, 1.02–2.30) and left ventricular outflow tract gradient progression (hazard ratio, 1.45; 95% confidence interval, 1.31–1.62). Additional myectomy did not reduce the risk for reoperation (P=0.92) but significantly increased the risk of a complete heart block requiring pacemaker implantation (8.1% versus 1.7%; P=0.005). Conclusions— Survival is excellent after surgery for discrete subaortic stenosis; however, reoperation for recurrent discrete subaortic stenosis is not uncommon. Over time, the left ventricular outflow tract gradient slowly increases and mild aortic regurgitation is common, although generally nonprogressive over time. Myectomy does not show additional advantages, and because it is associated with an increased risk of complete heart block, it should not be performed routinely.

Aortic stenosis at young adult age

Aortic stenosis at young adult age is usually the result of a stenotic bicuspid aortic valve, which is the most common cardiac congenital anomaly. In clinical practice, exercise and pregnancy are important topics. Furthermore, the timing of intervention is under debate, as little information is available on the natural history and outcome after aortic valve replacement in these young adults. In older patients, there is a trend towards earlier intervention. With the increased knowledge of the pathophysiology of aortic stenosis, studies have focused on the dilatation of the ascending aorta with risk of dissection. Recently, it has been suggested that pharmacologic treatment of aortic stenosis could be beneficial for these young adults.

Ascending aorta dilatation in patients with bicuspid aortic valve stenosis: a prospective CMR study

AbstractBackgroundThe aim of this study was to evaluate the natural progression of aortic dilatation and its association with aortic valve stenosis (AoS) in patients with bicuspid aortic valve (BAV).MethodsProspective study of aorta dilatation in patients with BAV and AoS using cardiac magnetic resonance (CMR). Aortic root, ascending aorta, aortic peak velocity, left ventricular systolic and diastolic function and mass were assessed at baseline and at 3-year follow-up.ResultsOf the 33 enrolled patients, 5 needed surgery, while 28 patients (17 male; mean age: 31u2009±u20098xa0years) completed the study. Aortic diameters significantly increased at the aortic annulus, sinus of Valsalva and tubular ascending aorta levels (Pu2009<u20090.050). The number of patients with dilated tubular ascending aortas increased from 32xa0% to 43xa0%. No significant increase in sino-tubular junction diameter was observed. Aortic peak velocity, ejection fraction and myocardial mass significantly increased while the early/late filling ratio significantly decreased at follow-up (Pu2009<u20090.050). The progression rate of the ascending aorta diameter correlated weakly with the aortic peak velocity at baseline (R2u2009=u20090.16, Pu2009=u20090.040).ConclusionBAV patients with AoS showed a progressive increase of aortic diameters with maximal expression at the level of the tubular ascending aorta. The progression of aortic dilatation correlated weakly with the severity of AoS.Key Points• Bicuspid aortic valve (BAV) is the most common congenital heart defect.n • BAV patients have an increased risk of developing aortic valve stenosis (AoS).n • BAV patients have an increased risk of developing thoracic aorta dilatation.n • The severity of aortic stenosis is correlated to the progression of aortic dilatation.n • Cardiac magnetic resonance can rapidly assess patients with a bicuspid aortic valve.

Non-sustained ventricular tachycardia in patients with congenital heart disease: An important sign?

BACKGROUNDnSustained ventricular tachycardia (susVT) and ventricular fibrillation (VF) are observed in adult patients with congenital heart disease (CHD). These dysrhythmias may be preceded by non-sustained ventricular tachycardia (NSVT). The aims of this study are to examine the 1] time course of ventricular tachyarrhythmia (VTA) in a large cohort of patients with various CHDs and 2] the development of susVT/VF after NSVT.nnnMETHODSnIn this retrospective study, patients with VTA on ECG, 24-hour Holter or ICD-printout or an out-of-hospital-cardiac arrest due to VF were included. In patients with an ICD, the number of shocks was studied.nnnRESULTSnPatients (N=145 patients, 59% male) initially presented with NSVT (N=103), susVT (N=25) or VF (N=17) at a mean age of 40 ± 14 years. Prior to VTA, 58 patients had intraventricular conduction delay, 14 an impaired ventricular dysfunction and 3 had coronary artery disease. susVT/VF rarely occurred in patients with NSVT (N=5). Fifty-two (36%) patients received an ICD; appropriate and inappropriate shocks, mainly due to supraventricular tachycardia (SVT), occurred in respectively 15 (29%) (NSVT: N=1, susVT: N=9, VF: N=5) and 12 (23%) (NSVT: N=4, susVT: N=5, VF: N=3) patients.nnnCONCLUSIONSnVTA in patients with CHD appear on average at the age of 40 years. susVT/VF rarely developed in patients with only NSVT, whereas recurrent episodes of susVT/VF frequently developed in patients initially presenting with susVT/VF. Hence, a wait-and-see treatment strategy in patients with NSVT and aggressive therapy of both episodes of VTA and SVT in patients with susVT/VF seems justified.