Biatrial arrhythmogenic substrate in patients with hypertrophic obstructive cardiomyopathy.

BACKGROUND: Atrial fibrillation (AF) in patients with hypertrophic obstructive cardiomyopathy (HOCM) may be caused by a primary atrial myopathy. Whether HOCM-related atrial myopathy affects mainly electrophysiological properties of the left atrium (LA) or also the right atrium (RA) has never been investigated. OBJECTIVE: The purpose of this study was to characterize atrial conduction and explore differences in the prevalence of conduction disorders, potential fractionation, and low-voltage areas (LVAs) between the RA and LA during sinus rhythm (SR) as indicators of potential arrhythmogenic areas. METHODS: Intraoperative epicardial mapping of both atria during SR was performed in 15 HOCM patients (age 50 ± 12 years). Conduction delay (CD) and conductin block (CB), unipolar potential characteristics (voltages, fractionation), and LVA were quantified. RESULTS: Conduction disorders and LVA were found scattered throughout both atria in all patients and did not differ between the RA and LA (CD: 2.9% [1.9%-3.6%] vs 2.6% [2.1%-6.4%], P = .541; CB: 1.7% [0.9%-3.1%] vs 1.5% [0.5%-2.8%], P = .600; LVA: 4.7% [1.6%-7.7%] vs 2.9% [2.1%-7.1%], P = .793). Compared to the RA, unipolar voltages of single potentials (SPs) and fractionated potentials (FPs) were higher in the LA (SP: P75 7.3 mV vs 10.9 mV; FP: P75 2.0 mV vs 3.7 mV). FP contained low-voltage components in only 18% of all LA sites compared to 36% of all RA sites. CONCLUSION: In patients with HOCM, conduction disorders, LVA, and FP are equally present in both atria, supporting the hypothesis of a primary atrial myopathy. Conceptually, the presence of a biatrial substrate and high-voltage FP may contribute to failure of ablative therapy of atrial tachyarrhythmias in this population.

Biomechanical response of ultrathin slices of hypertrophic cardiomyopathy tissue to myosin modulator mavacamten.

Hypertrophic cardiomyopathy (HCM) is the most common inherited myocardial disorder of the heart, but effective treatment options remain limited. Mavacamten, a direct myosin modulator, has been presented as novel pharmacological therapy for HCM. The aim of this study was to analyze the biomechanical response of HCM tissue to Mavacamten using living myocardial slices (LMS). LMS (n = 58) from patients with HCM (n = 10) were cultured under electromechanical stimulation, and Verapamil and Mavacamten were administered on consecutive days to evaluate their effects on cardiac biomechanics. Mavacamten and Verapamil reduced contractile force and dF/dt and increased time-to-relaxation in a similar manner. Yet, the time-to-peak of the cardiac contraction was prolonged after administration of Mavacamten (221.0 ms (208.8 - 236.3) vs. 237.7 (221.0 - 254.7), p = 0.004). In addition, Mavacamten prolonged the functional refractory period (FRP) (330 ms (304 - 351) vs. 355 ms (313 - 370), p = 0.023) and better preserved twitch force with increasing stimulation frequencies, compared to Verapamil. As such, Mavacamten reduced (hyper-)contractility and prolonged contraction duration of HCM LMS, suggesting a reduction in cardiac wall stress. Also, Mavacamten might protect against the development of ventricular tachyarrhythmias due to prolongation of the FRP, and improve toleration of tachycardia due to better preservation of twitch force at tachycardiac stimulation frequencies.

In-vivo Sino-Atrial Node Mapping in Children and Adults With Congenital Heart Disease.

Background: Sinus node dysfunction (SND) and atrial tachyarrhythmias frequently co-exist in the aging patient with congenital heart disease (CHD), even after surgical correction early in life. We examined differences in electrophysiological properties of the sino-atrial node (SAN) area between pediatric and adult patients with CHD. Methods: Epicardial mapping of the SAN was performed during sinus rhythm in 12 pediatric (0.6 [0.4-2.4] years) and 15 adult (47 [40-55] years) patients. Unipolar potentials were classified as single-, short or long double- and fractionated potentials. Unipolar voltage, relative R-to-S-amplitude ratio and duration of all potentials was calculated. Conduction velocity (CV) and the amount of conduction block (CB) was calculated. Results: SAN activity in pediatric patients was solely observed near the junction of the superior caval vein and the right atrium, while in adults SAN activity was observed even up to the middle part of the right atrium. Compared to pediatric patients, the SAN region of adults was characterized by lower CV, lower voltages, more CB and a higher degree of fractionation. At the earliest site of activation, single potentials from pediatrics consisted of broad monophasic S-waves with high amplitudes, while adults had smaller rS-potentials with longer duration which were more often fractionated. Conclusions: Compared to pediatric patients, adults with uncorrected CHD have more inhomogeneous conduction and variations in preferential SAN exit site, which are presumable caused by aging related remodeling. Long-term follow-up of these patients is essential to demonstrate whether these changes are related to development of SND and also atrial tachyarrhythmias early in life.

Impact of sex on timing and clinical outcome of septal myectomy for obstructive hypertrophic cardiomyopathy.

BACKGROUND: Sex disparities are common in hypertrophic cardiomyopathy (HCM). Previous research has shown that at time of myectomy, women are older, have greater impairment of diastolic function and more advanced cardiac remodeling. The clinical impact of these differences is unknown. METHOD: This study included 162 HCM patients (61% men) who underwent septal myectomy. Time to treatment was calculated in relation to symptom onset and diagnosis. Pre- and post-operative echocardiographic data were collected. Sex differences were assessed at baseline and in time-to-event survival analyses for the composite endpoint of all-cause mortality, cardiac transplantation, re-intervention and aborted sudden cardiac death. RESULTS: Women were generally older at time of myectomy (57 vs. 49 years, p < 0.01), with similar time to treatment as measured from symptom onset (2.3 [1.3-6.0] vs. 2.8 [1.1-5.3] years, p > 0.05), but a shorter time since diagnosis compared to men (2.6 [1.2-7.0] vs. 4.3 [2.4-8.3] years, p = 0.02). Mean wall thickness and left atrial diameter were the same for men and women, but were higher in women when correcting for body surface area (absolute: 20 vs. 19 mm, 48 vs 46 mm, p ≥ 0.05; corrected: 9.7 vs. 11.2 mm/m2, 23.4 vs. 26.3 mm/m2, p < 0.01). After 5.9 [3.0-9.1] years, 15% of men and 8% of women had reached the composite endpoint (p > 0.05). CONCLUSION: In conclusion, although women present later in life and seem to have more advanced disease on echocardiography, time until myectomy was similar and clinical outcomes after myectomy are favourable for both men and women.

Early and late post-operative arrhythmias after surgical myectomy: 45 years of follow-up.

AIMS: The aims of this study are to investigate the incidence and determinants of post-operative atrial arrhythmias, conduction disorders and mortality in hypertrophic obstructive cardiomyopathy (HOCM) patients undergoing transaortic myectomy. METHODS AND RESULTS: This retrospective single-center study was conducted in 249 patients (median age 54 years [40-64], 42% female) undergoing transaortic myectomy. Post-operative atrial fibrillation (AF) was reported in 84 patients (33.7%), including 56 patients (22.5%) with de novo AF. Older age (HR = 1.027 (1.003-1.052), p = 0.029) and hypercholesterolemia (HR = 2.296 (1.091-4.832) p = 0.029) were independent predictors for de novo post-operative AF. Late post-operative AF and atrial flutter (AFL) occurred in 18.9% and 6.8% of the patients, respectively. De novo early post-operative AF increased the risk of late post-operative AF (HR = 3.138 (1.450-6.789), p = 0.004). Patients with a right bundle branch block had a higher risk of early-postoperative pacemaker implantation (p = 0.003, HR = 9.771 (2.195-43.505)). Higher age at time of surgery (HR = 1.053 (1.026-1.081), p < 0.001) was a predictor for late mortality (n = 47, 18.9%). CONCLUSION: Early and late post-operative AF, AFL and other SVTs are common sequelae after myectomy and are associated with older age at surgery, history of AF and early post-operative AF. Early post-operative arrhythmias are not transient and periodic rhythm monitoring is therefore essential to initiate therapy as soon as possible.

Protein Quality Control Activation and Microtubule Remodeling in Hypertrophic Cardiomyopathy.

Hypertrophic cardiomyopathy (HCM) is the most common inherited cardiac disorder. It is mainly caused by mutations in genes encoding sarcomere proteins. Mutant forms of these highly abundant proteins likely stress the protein quality control (PQC) system of cardiomyocytes. The PQC system, together with a functional microtubule network, maintains proteostasis. We compared left ventricular (LV) tissue of nine donors (controls) with 38 sarcomere mutation-positive (HCMSMP) and 14 sarcomere mutation-negative (HCMSMN) patients to define HCM and mutation-specific changes in PQC. Mutations in HCMSMP result in poison polypeptides or reduced protein levels (haploinsufficiency, HI). The main findings were 1) several key PQC players were more abundant in HCM compared to controls, 2) after correction for sex and age, stabilizing heat shock protein (HSP)B1, and refolding, HSPD1 and HSPA2 were increased in HCMSMP compared to controls, 3) α-tubulin and acetylated α-tubulin levels were higher in HCM compared to controls, especially in HCMHI, 4) myosin-binding protein-C (cMyBP-C) levels were inversely correlated with α-tubulin, and 5) α-tubulin levels correlated with acetylated α-tubulin and HSPs. Overall, carrying a mutation affects PQC and α-tubulin acetylation. The haploinsufficiency of cMyBP-C may trigger HSPs and α-tubulin acetylation. Our study indicates that proliferation of the microtubular network may represent a novel pathomechanism in cMyBP-C haploinsufficiency-mediated HCM.

Tetralogy of Fallot in the Current Era.

Only few studies have reported long-term outcome of the transatrial-transpulmonary approach in the current era of management of tetralogy of Fallot (ToF). We investigated 15-year outcome of correction via a transatrial-transpulmonary approach in a large cohort of successive patients operated in the 21st century. All infant ToF patients undergoing transatrial-transpulmonary ToF correction between 2000 and 2015 were included (N = 177, 106 male, median follow-up 7.1 (interquartile range 3.0-10.9) years. Data regarding postoperative complications, reinterventions, development of atrial and ventricular arrhythmia, cardiac function, and survival were evaluated. Prior shunting was performed in 10 patients (6%). The transatrial-transpulmonary approach resulted in valve-sparing surgery in 57 patients (32%). Postoperative surgical complications included junctional ectopic tachycardia (N = 12, 7%), pericardial (N = 10, 6%) or pleural effusion (N = 7, 3%), chylothorax (N = 7, 4%), bleeding requiring reoperation (N = 4, 3%), and superficial wound infection (N = 1). Fifty-one patients underwent 68 reinterventions, mainly due to pulmonary restenosis (PS) (N = 57). ToF correction at age <2 months and double outlet or double-chambered right ventricle variants of the ToF spectrum were independent predictors for reintervention. Patients undergoing valve-sparing ToF correction had a significant longer PR-free survival than those with a transannular patch (8.5 [95% confidence interval 6.8-10.3] years vs 1.1 [95% confidence interval 0.8-1.5] years; P < 0.001). Overall mortality was 2.8%; mortality rates were higher in premature/dysmature newborns (0.7% vs 9.5%; P < 0.001). Although the 15-year outcome of the transatrial-transpulmonary approach in terms of postoperative complications and mortality rates is excellent, the high incidence of moderate and severe PR is worrisome. Valve-sparing surgery was associated with a substantially lower incidence of PR, yet was surgically not possible in the majority of patients.

Surgical correction of a coronary fistula between the left main coronary artery and the coronary sinus in a neonate.

We present a rare case of a 6-day old female neonate with a particularly exceptional coronary anatomy encompassing a fistula between the left main coronary artery and the coronary sinus with steal phenomenon, mandating surgical correction. The condition is extremely rare with an estimated incidence of about 0.1-0.2%. We operated this girl early on to prevent ischaemic events and pulmonary overflow with future heart failure. Further, we discuss possible aetiology and pathophysiological mechanisms of coronary artery fistulae and steal phenomena. Depending on specific anatomic characteristics, an interventional approach may be an alternative for surgical closure; however, this was not possible in our case.

Outcome after aortic valve replacement in children: A systematic review and meta-analysis.

OBJECTIVE: Despite an increasing interest in pediatric aortic valve repair, aortic valve replacement in children may be unavoidable. The evidence on outcome after pediatric aortic valve replacement is limited and usually reported in small case series. This systematic review and meta-analysis aims to provide an overview of reported outcome of pediatric patients after aortic valve replacement. METHODS: A systematic literature search for publications reporting outcome after pediatric aortic valve replacement published between January 1990 and May 2015 was conducted. Studies written in English with a study size of more than 30 patients were included. RESULTS: Thirty-four publications reporting on 42 cohorts were included in this review: 26 concerning the Ross procedure (n = 2409), 13 concerning mechanical prosthesis aortic valve replacement (n = 696), and 3 concerning homograft aortic valve replacement (n = 224). There were no studies on bioprostheses that met our inclusion criteria. The pooled mean patient age was 9.4 years, 12.8 years, and 8.9 years for Ross, mechanical prosthesis, and homograft recipients, respectively. Pooled mean follow-up was 6.6 years. The Ross procedure was associated with lower early (4.20%; 95% confidence interval [CI], 3.37-5.22 vs 7.34%; 95% CI, 5.21-10.34 vs 12.82%; 95% CI, 8.91-18.46) and late mortality (0.64%/y; 95% CI, 0.49-0.84 vs 1.23%/y; 95% CI, 0.85-1.79 vs 1.59%/y; 95% CI, 1.03-2.46) compared with mechanical prosthesis aortic valve replacement and homograft aortic valve replacement, respectively. No significantly different aortic valve reoperation rates were observed between the Ross procedure and mechanical prosthesis aortic valve replacement (1.60%/y; 95% CI, 1.27-2.02 vs 1.07%/y; 95% CI, 0.68-1.68, respectively), whereas homograft aortic valve replacement was associated with significantly higher aortic valve reoperation rates (5.44%/y; 95% CI, 4.24-6.98). The Ross procedure-associated right ventricular outflow tract reoperation rate was 1.91% per year (95% CI, 1.50-2.44). CONCLUSIONS: This systematic review illustrates that all currently available aortic valve substitutes are associated with suboptimal results in children, reflecting the urgent need for reliable and durable repair techniques and innovative replacement solutions for this challenging group of patients.

Long-term benefit of myectomy and anterior mitral leaflet extension in obstructive hypertrophic cardiomyopathy.

Severely symptomatic patients with obstructive hypertrophic cardiomyopathy (HC) may benefit from surgical myectomy. In patients with enlarged mitral leaflets and mitral regurgitation, myectomy can be combined with anterior mitral leaflet extension (AMLE) to stiffen the midsegment of the leaflet. The aim of this study was to evaluate the long-term results of myectomy combined with AMLE in patients with obstructive HC. This prospective, observational, single-center cohort study included 98 patients (49 ± 14 years, 37% female) who underwent myectomy combined with AMLE from 1991 to 2012. End points included all-cause mortality and change in clinical and echocardiographic characteristics. Mortality was compared with age- and gender-matched patients with nonobstructive HC and subjects from the general population. Long-term follow-up was 8.3 ± 6.1 years. There was no operative mortality, and New York Heart Association class was reduced from 2.8 ± 0.5 to 1.3 ± 0.5 (p <0.001), left ventricular outflow tract gradient from 93 ± 25 to 9 ± 8 mm Hg (p <0.001), mitral valve regurgitation from grade 2.0 ± 0.9 to 0.5 ± 0.8 (p <0.001), and systolic anterior motion of the mitral valve from grade 2.4 ± 0.9 to 0.1 ± 0.3 (p <0.001). The 1-, 5-, 10-, and 15-year cumulative survival rates were 98%, 92%, 86%, and 83%, respectively, and did not differ from the general population (99%, 97%, 92%, and 85%, respectively, p = 0.3) or patients with nonobstructive HC (98%, 97%, 88%, and 83%, respectively, p = 0.8). In conclusion, in selected patients with obstructive HC, myectomy combined with AMLE is a low-risk surgical procedure. It results in long-term symptom relief and survival similar to the general population.

Carcinoid heart disease: outcomes after surgical valve replacement.

OBJECTIVES: To describe the early and late outcomes of carcinoid patients undergoing surgical heart valve replacement. METHODS: In a retrospective study, records of patients with symptomatic carcinoid heart disease referred for valve surgery between 1993 and 2010 at two academic centres were reviewed. The perioperative and postoperative outcomes were analysed. RESULTS: Nineteen patients, with a mean age of 56 ± 9.6 years, underwent cardiac surgery for carcinoid syndrome. Sixteen patients underwent implantation of one or more mechanical bileaflet valve prosthesis and three patients had one or more bioprosthetic valves implanted. Survival after 1 and 5 years was 71 and 43%, respectively. Six out of nine survivors were at last follow-up in New York Heart Association class I. Valve-related events such as valve thrombosis or bleeding complications were not registered. Echocardiography showed improvement of right ventricular dilatation in 80% of patients. CONCLUSIONS: Despite advanced cardiac morbidity at the time of operation, early postoperative survival was 90%. Long-term survival of patients with carcinoid heart disease undergoing valve replacement is determined by carcinoid progression. The surviving patients had a persistent improvement in functional capacity without valve-related complications of the mechanical prosthesis.

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

BACKGROUND: In 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. METHODS: Between 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. RESULTS: Hospital 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. CONCLUSIONS: Right 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.

Relevance of colloid oncotic pressure regulation during neonatal and infant cardiopulmonary bypass: a prospective randomized study.

OBJECTIVE: In neonatal and infant cardiac surgery with cardiopulmonary bypass (CPB), hemodilution with reduction of plasma albumin concentration and low colloid oncotic pressure (COP) are the main factors associated with tissue edema and postoperative weight gain. The aim of our study was to evaluate the influence of two different COP regulatory strategies on post-bypass body weight gain, fluid balance, and clinical outcomes. METHODS: Seventy elective patients with body weight<10 kg underwent first-time cardiac surgery with CPB and were randomized into two groups. The standard COP group received 0.5 g kg(-1) of human albumin in the priming and, during CPB, albumin was added to maintain the COP>15 mmHg. In the high COP group, albumin concentration in the priming was 5% and, during CPB, the COP was maintained above 18 mmHg. All patients were monitored before, during and until 24h postoperatively. Data were collected on body weight gain, COP, albumin concentration, fluids transfusion, blood loss, urine production and laboratory results. RESULTS: Patients' demographics and operative data were comparable. Although the high COP group had perioperatively significantly higher COP and albumin concentration than the standard COP group, no significant difference was found in the body weight gain. There were also no significant differences between the groups with respect to fluid balance, urine output and blood loss. However, the high COP group had significantly shorter postoperative duration of mechanical ventilation (10h vs 14 h, p=0.02) and lower plasma lactate concentration post operation (1.1 mmoll(-1) vs 1.4 mmoll(-1), p=0.046). CONCLUSIONS: The COP regulatory strategy for neonatal and infant CPB, based upon the 5% concentration of albumin in the priming and a COP target of 18 mmHg during bypass, better preserves the plasma albumin concentration within the physiological range and stabilizes the colloid pressure than the standard strategy (0.5 gkg(-1) albumin in the priming and bypass COP target at 15 mmHg). Nevertheless, only the lower postoperative plasma lactate concentration and the shorter duration of mechanical ventilation in the high COP group indicated the potential clinical benefit of this new strategy.

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

OBJECTIVE: Allograft 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. METHODS: Between 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. RESULTS: The 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. CONCLUSIONS: Right-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.

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 STUDY: To 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. METHODS: A 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. RESULTS: Excluding 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. CONCLUSIONS: Long 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.

Long-term outcome of alcohol septal ablation in patients with obstructive hypertrophic cardiomyopathy: a word of caution.

BACKGROUND: The impact of alcohol septal ablation (ASA)-induced scar is not known. This study sought to examine the long-term outcome of ASA among patients with obstructive hypertrophic cardiomyopathy. METHODS AND RESULTS: Ninety-one consecutive patients (aged 54+/-15 years) with obstructive hypertrophic cardiomyopathy underwent ASA. Primary study end point was a composite of cardiac death and aborted sudden cardiac death including appropriate cardioverter-defibrillator discharges for fast ventricular tachycardia/ventricular fibrillation. Secondary end points were noncardiac death and other nonfatal complications. Outcomes of ASA patients were compared with 40 patients with hypertrophic cardiomyopathy who underwent septal myectomy. During 5.4+/-2.5 years, primary and/or secondary end points were seen in 35 (38%) ASA patients of whom 19 (21%) patients met the primary end point. The 1-, 5-, and 8-year survival-free from the primary end point was 96%, 86%, and 67%, respectively in ASA patients versus 100%, 96%, and 96%, respectively in myectomy patients during 6.6+/-2.7 years (log-rank, P=0.01). ASA patients had a approximately 5-fold increase in the estimated annual primary end point rate (4.4% versus 0.9%) compared with myectomy patients. In a multivariable model including a propensity score, ASA was an independent predictor of the primary end point (unadjusted hazard ratio, 5.2; 95% CI, 1.2 to 22.1; P=0.02 and propensity score-adjusted hazard ratio, 6.1; 95% CI, 1.4 to 27.1; P=0.02). CONCLUSIONS: This study shows that ASA has potentially unwanted long-term effects. This poses special precaution, given the fact that ASA is practiced worldwide at increasing rate. We recommend myectomy as the preferred intervention in patients with obstructive hypertrophic cardiomyopathy.

Alveolar recruitment strategy and PEEP improve oxygenation, dynamic compliance of respiratory system and end-expiratory lung volume in pediatric patients undergoing cardiac surgery for congenital heart disease.

OBJECTIVE: Optimizing alveolar recruitment by alveolar recruitment strategy (ARS) and maintaining lung volume with adequate positive end-expiratory pressure (PEEP) allow preventing ventilator-induced lung injury (VILI). Knowing that PEEP has its most beneficial effects when dynamic compliance of respiratory system (Crs) is maximized, we hypothesize that the use of 8 cm H(2)O PEEP with ARS results in an increase in Crs and end-expiratory lung volume (EELV) compared to 8 cm H(2)O PEEP without ARS and to zero PEEP in pediatric patients undergoing cardiac surgery for congenital heart disease. METHODS: Twenty consecutive children were studied. Three different ventilation strategies were applied to each patient in the following order: 0 cm H(2)O PEEP, 8 cm H(2)O PEEP without an ARS, and 8 cm H(2)O PEEP with a standardized ARS. At the end of each ventilation strategy, Crs, EELV, and arterial blood gases were measured. RESULTS: EELV, Crs, and P(a)O(2)/FiO(2) ratio changed significantly (P < 0.001) with the application of 8 cm H2O + ARS. Mean P(a)CO(2)- PETCO(2) difference between 0 PEEP and 8 cm H2O PEEP + ARS was also significant (P < 0.05). CONCLUSION: An alveolar recruitment strategy with relative high PEEP significantly improves Crs, oxygenation, P(a)CO(2)- PETCO(2) difference, and EELV in pediatric patients undergoing cardiac surgery for congenital heart disease.

Rapid method for intraoperative assessment of aortic coarctation using three-dimensional echocardiography.

AIMS: The availability of three-dimensional (3D) echography with its multiplanar review analysis software on board now allows detailed examination in assessing morphological details. We evaluated the feasibility of 3D echography in assessing intraoperative morphological details of aortic coarctation (CoA) and its repair. METHODS AND RESULTS: Nine consecutive children scheduled for surgery of CoA were intraoperatively evaluated. Intraoperative 3D data sets were taken and analysed online before resection of the coarctation, showing the cross-sectional area (CSA) of the proximal aorta, coarctation, and the distal descending aorta. After resection of the coarctation and extended end-to-end anastomosis, a 3D data set was recorded to analyse the CSA of the anastomosis. In nine out of nine consecutive procedures, intraoperative 3D echography permitted comprehensive viewing and measuring of CoA and its repair. In three out of nine surgical procedures, intraoperative 3D echography provided additional information to support surgical decision-making. CONCLUSION: Intraoperative 3D echography is a feasible non-invasive imaging modality for intraoperative assessment of CoA and its repair, which provides useful additional information.

Outcome after reoperation for atrioventricular septal defect repair.

Results of surgical repair of atrioventricular septal defect (AVSD), both partial (PAVSD) and complete (CAVSD), have improved. However, reoperation is not uncommon. This report describes our experience in 59 patients who underwent reoperation after AVSD repair, between 1977 and 2008. Thirty-one patients had a PAVSD, 28 had a CAVSD. Mean interval between initial repair and reoperation was 10+/-11 years (PAVSD vs. CAVSD: 13+/-12 vs. 6+/-9 years, P=0.063). Reoperations were required for left atrioventricular valve regurgitation (LAVVR) in 53 patients (combined with right atrioventricular valve regurgitation in 10, atrial septal defect (ASD) in 11, ventricular septal defect (VSD) in 7, left ventricular outflow tract (LVOT) obstruction in 1, and aortic valve stenosis in 1), ASD in 3, and LVOT obstruction in 3. Valve repair was performed in 45 patients and replacement in 8. Repair techniques of the left-sided atrioventricular valve (LAVV) included cleft closure in 44 patients, commissuroplasty in 19, and annuloplasty in 1. Freedom from additional reoperation was 85%, and 80% at 5 and 15 years. Hospital mortality was 3%. Overall survival was 91%, and 86% after 5 and 15 years. The most common indication to undergo reoperation is LAVVR. Reoperation is safe and in the majority of cases, a durable repair of the LAVV can still be achieved.