B.J. Bouma

Contemporary survival of adults with congenital heart disease

Background Survival data that are applicable to the current population of adults with congenital heart disease (CHD) are not available. Objectives Using an alternative survival analysis with age as the primary time scale, we assessed the contemporary survival of adult patients with CHD. Methods Survival was assessed using prospective data of the national registry of adult patients with CHD of the Netherlands. Survival was stratified by severity and lesion, and compared with a standardised general population. Results Mean age at inclusion was 37u2005years, and 49% of the study population was male. During a cumulative prospective follow-up of 90u2005270 patient-years in 14u2005327 patients, 535 deaths occurred. Median survival was 53.4 (95% CI 49.9 to 60.7), 75.4 (95% CI 72.9 to 79.1) and 84.1 (95% CI 81.9 to 87.0) years for patients with severe, moderate and mild lesions, respectively. Survival of most patients with mild lesions did not differ from the general population, while, as expected, survival of patients with severe and moderate lesions was substantially lower (<0.001). Conclusions The present study gives insight in the contemporary survival of adults with CHD. This may aid patient counselling, timing of interventions and future research.

Persistent neo-aortic growth during adulthood in patients after an arterial switch operation

Objective After the arterial switch operation (ASO), disproportional neo-aortic growth during childhood has been reported. Even though it has been suggested neo-aortic dilation will stabilise in adulthood, data are lacking. The aim of this study was to assess the change in neo-aortic dimensions, prevalence of neo-aortic dilation >40u2005mm and long-term outcome in adults who underwent ASO in childhood. Methods All 116 ASO patients operated in a tertiary referral centre and born before 1995 were included. Of these, 83 (72%) survived to adulthood (>17u2005years) and six were lost to follow-up. Neo-aortic measurements performed in adulthood were collected from available echocardiographic, cardiovascular magnetic resonance and CT images. The time trend was analysed using a mixed model, adjusted for imaging modality. Results Clinical data with at least one measurement of the neo-aortic diameter were available in 77 (93%) adult patients and serial measurements in 65 (78%). At baseline (median age 18.1u2005years), mean neo-aortic diameter was 36±5u2005mm. Mean neo-aortic growth was 0.31u2005mm/year (p<0.001 compared with normal value 0.08u2005mm/year) and was linear over time. Freedom from neo-aortic dilation beyond a diameter of 40u2005mm was 23% at 28u2005years of age. During a mean clinical follow-up in adulthood of 7.2u2005years (IQR 4.0 to 10.1), 3 (4%) patients underwent neo-aortic replacement. No other neo-aortic complications occurred. Conclusions In early adulthood, neo-aortic growth was on average linear and did not stabilise over time.

Advanced therapy for pulmonary arterial hypertension due to congenital heart disease: a clinical perspective in a new therapeutic era

IntroductionPulmonary arterial hypertension (PAH) is a progressivedisease with poor survival. PAH is classified by the 2009updated clinical classification of pulmonary hypertensionand a major subgroup is PAH due to congenital heartdisease (CHD) with systemic-to-pulmonary shunt [1]. Thisshunting may lead to extensive histological changes in thedistal pulmonary arteries resulting in an irreversibleincrease in pulmonary vascular resistance (PVR). Theclassification of CHD-PAH includes four categories: (1)Eisenmenger syndrome, (2) PAH associated with systemic-to-pulmonary shunts, (3) PAH with small septal defects,and (4) PAH after corrective cardiac surgery [2]. In thesepatients advanced pharmacological therapy should beconsidered.Three classes of advanced therapy for PAH are currentlyin use: prostanoids such as epoprostenol, endothelin-1receptor antagonists such as bosentan, and phosphodiester-ase 5 inhibitors such as sildenafil [3]. Bosentan has beenshown to improve 6-min walking distance (6MWD) and todecrease PVR in patients with Eisenmenger syndrome [4].Anecdotal experience with the phosphodiesterase type-5inhibitors sildenafil and tadalafil show favourable function-al and haemodynamic results in patients with CHD-PAH[5, 6]. We present three patients treated with advancedpharmacological therapy for CHD-PAH: a patient withEisenmenger syndrome receiving standard care, a patientwith atrial septal defect receiving advanced therapy as abridge to surgery, and a patient with segmental PAH whowas started on advanced therapy empirically.Case 1: an Eisenmenger patientThe first case describes a 38-year-old male patient with adouble inlet left ventricle, hypoplastic aortic arch and patentductus arteriosus. He was deemed ineligible for a Fontancirculation and developed PAH in childhood. He wasseverely symptomatic (NYHA functional class III-IV) andcould only live a sedentary lifestyle. He had been on thewaiting list for combined heart and lung transplantation for2 years. He was cyanotic with a peripheral saturation of80%. His haemoglobin level was 13.4 mmol/L. Onauscultation, normal heart sounds were heard, as well as asystolic murmur grade II/VI. Trans-thoracic echocardiogra-phy (TTE) showed a moderate systolic ventricular function,mild mitral regurgitation, and moderate tricuspid regurgita-

Bosentan in pulmonary arterial hypertension: a comparison between congenital heart disease and chronic pulmonary embolism.

Background. In patients with pulmonary hypertension, it is unknown whether the treatment effect of bosentan is dependent on the duration of pulmonary vessel changes. Therefore, we studied the response to bosentan in patients with life-long pulmonary vessel changes (pulmonary arterial hypertension (PAH) due to congenital heart disease (CHD)) and in patients with subacutely induced pulmonary vessel changes (chronic thromboembolic pulmonary hypertension (CTEPH)).Methods. In this open-label study, 18 patients with PAH due to CHD and 16 patients with CTEPH were treated with bosentan for at least one year. All patients were evaluated at baseline and during follow-up by means of the six-minute walk distance (6-MWD) and laboratory tests.Results. Improvement of 6-MWD was comparable in patients with PAH due to CHD (444±112 m to 471±100 m, p=0.02), and in CTEPH (376±152 m to 423±141 m, p=0.03) after three months of treatment. After this improvement, 6-MWD stabilised in both groups.Conclusion. Although duration of pulmonary vessel changes is strikingly different in patients with PAH due to CHD and CTEPH, the effect of one year of bosentan treatment was comparable. The main treatment effect appears to be disease stabilisation and decreasing the rate of deterioration. (Neth Heart J 2009;17:334–8.)

Is cardiac CT a reproducible alternative for cardiac MR in adult patients with a systemic right ventricle

Objective20xa0% of patients with a systemic RV are pacemaker dependent, and unsuitable to undergo cardiac magnetic resonance (CMR). Multidetector row computed tomography (MDCT) could provide a reproducible alternative to CMR in these patients. The aim of this study was to compare variability of MDCT with CMR.MethodsThirty-five patients with systemic RV underwent either MDCT (nu2009=u200915) or CMR (nu2009=u200920). Systemic RV volumes and ejection fraction were obtained, and intra- and interobserver variability for both modalities were assessed and compared.ResultsWe found the intra- and interobserver variability of volumes and function measurements of the systemic RV obtained with MDCT to be higher compared with those obtained with CMR. However, these differences in variability were not significant, the only exception being the interobserver variability of systemic RV stroke volume.ConclusionsMDCT provides a reproducible alternative to CMR for volumes and function assessment in patients with a systemic RV.

Mortality in pulmonary arterial hypertension due to congenital heart disease: Serial changes improve prognostication

BACKGROUNDnAdult patients with pulmonary arterial hypertension due to congenital heart disease (PAH-CHD) suffer from high mortality. This underlines the importance of adequate risk stratification to guide treatment decisions. Several baseline parameters are associated with mortality, however, their prognostic value may weaken after years of follow-up. Therefore we investigated the prognostic value of serial changes in standard clinical parameters in PAH-CHD.nnnMETHODSnIn this prospective observational cohort study we included consecutive PAH-CHD adults, between 2005 and 2016. Control visits to the outpatient clinic were standardized, including functional, biochemical and echocardiographic tests, according to the guidelines. The prognostic value of serial changes was determined with time-dependent Cox regression.nnnRESULTSnNinety-two patients with PAH-CHD were included (age 43±15years, 34% male, 38% Down, 73% Eisenmenger). During a median follow-up of 6.0 (IQR 3.7-9.3) years, 35 (38%) patients died. Serial changes in World Health Organization functional classification (WHO-FC, HR 18.34 for onset class IV), six-minute walk distance (6-MWD, HR 0.65 per 50m), oxygen saturation at peak exercise (peak SaO2, HR 0.74 per 5%), NTproBNP (HR 2.25 per 1000ng/l) and echocardiographic right ventricular function (TAPSE, HR 0.80 per 0.5cm) significantly predicted mortality. Moreover, serial changes in these parameters were more potent predictors compared to baseline parameters, based on reduction in -2 log likelihood.nnnCONCLUSIONSnSerial changes in standard clinical parameters have more prognostic value compared to baseline parameters in PAH-CHD. Our results emphasize the importance of screening for serial changes since periodical assessment could guide treatment decisions to delay disease progression.

Coronary anatomy as related to bicuspid aortic valve morphology

Objective Variable coronary anatomy has been described in patients with bicuspid aortic valves (BAVs). This was never specified to BAV morphology, and prognostic relevance of coronary vessel dominance in this patient group is unclear. The purpose of this study was to evaluate valve morphology in relation to coronary artery anatomy and outcome in patients with isolated BAV and with associated aortic coarctation (CoA). Methods Coronary anatomy was evaluated in 186 patients with BAV (141 men (79%), 51±14u2005years) by CT and invasive coronary angiography. Correlation of coronary anatomy was made with BAV morphology and coronary events. Results Strictly bicuspid valves (without raphe) with left-right cusp fusion (type 1B) had more left dominant coronary systems compared with BAVs with left-right cusp fusion with a raphe (type 1A) (48% vs. 26%, p=0.047) and showed more separate ostia (28% vs. 9%, p=0.016). Type 1B BAVs had more coronary artery disease than patients with type 1A BAV (36% vs. 19%, p=0.047). More left dominance was seen in BAV patients with CoA than in patients without (65% vs. 24%, p<0.05). Conclusions The incidence of a left dominant coronary artery system and separate ostia was significantly related to BAVs with left-right fusion without a raphe (type 1B). These patients more often had significant coronary artery disease. In patients with BAV and CoA, left dominancy is more common.

Beneficial effect of high dose statins on the vascular wall in patients with repaired aortic coarctation

BACKGROUNDnCarotid intima-media thickness (CIMT) is a marker for atherosclerosis. Adult post-coarctectomy patients (CoA) demonstrate an increased cardiovascular risk and increased CIMT compared to controls. This study evaluates the effect of high dose statins on the change in CIMT and cardiovascular risk.nnnMETHODSnWe designed a multicenter, prospective, randomized, open label trial with blinded endpoint (PROBE design) to evaluate the effect of three year treatment with atorvastatin 80 mg on CIMT and cardiovascular risk. Primary endpoint was CIMT measured by B mode ultrasonography. Secondary endpoints were mortality and morbidity due to cardiovascular disease and serum lipids.nnnRESULTSn155 patients (36.3 ± 11.8 years, 96 (62%) male) were randomized (atorvastatin=80, no treatment=75). There was no significant effect of atorvastatin on the change in CIMT (treatment effect -0.005, 95% CI, -0.039-0.029; P=0.76). A significant effect on serum cholesterol and LDL levels was found (- 0.71, 95% CI, - 1.16 to - 0.26; P = 0.002 vs - 0.66, 95% CI - 1.06 to - 0.26; P = 0.001). There was no difference in secondary outcome measures. Baseline CIMT was higher in hypertensive compared to normotensive CoA. (0.69 ± 0.16 mm vs 0.61 ± 0.98 mm; P=0.002). Hypertension (ß=0.043, P=0.031) was the strongest determinant CIMT.nnnCONCLUSIONnThree year treatment with atorvastatin does not lead to a reduction of CIMT and secondary outcome measures, despite a decrease in total cholesterol and LDL levels. Hypertensive CoA demonstrate the highest CIMT and the largest CIMT progression. Blood pressure control should be the main focus in CoA to decrease cardiovascular risk.

Per-operative stent placement in the right pulmonary artery; a hybrid technique for the management of pulmonary artery branch stenosis at the time of pulmonary valve replacement in adult Fallot patients

After having undergone surgical correction at an early age, many patients with tetralogy of Fallot develop long-term complications including progressive pulmonary regurgitation and peripheral pulmonary stenosis. A high percentage of these patients need to undergo a second operation in their adolescence or early adulthood. If simultaneous treatment of both pulmonary regurgitation and peripheral pulmonary stenosis is warranted, a complete surgical approach has several disadvantages. We describe four cases of Fallot patients with severe pulmonary regurgitation and peripheral pulmonary stenosis who were treated using a hybrid approach involving surgical implantation of a pulmonary homograft and peroperative stenting of the pulmonary artery.

Transvenous pacing after the Mustard procedure: considering the complications.

Disturbances of rhythm and conduction in patients undergoing surgery for transposition of the great arteries have been widely reported. Some of these patients require implantation of a permanent pacemaker, especially those in whom symptomatic sick sinus syndrome is diagnosed. We present the case of a 29-year-old male corrected with a Mustard procedure, who received a pacemaker for progressive atrioventricular conduction disturbances and sinus node dysfunction, and we review the possible complications associated with transvenous pacemaker implantation in these patients. (Neth Heart J 2007;15:387-389.)