Repair of atrioventricular disruption after mitral valve replacement utilizing commando procedure principles.

Atrioventricular rupture is a life-threatening complication of mitral valve replacement. We present how incising the intervalvular fibrosa critically improves exposure. The aortic valve sacrifice allows access to a large atrioventricular dissociation defect and reliable repair of the anterolateral aspect of mitral valve annulus.

Grown-up Congenital Heart Surgery in 1093 Consecutive Cases: A "Hidden" Burden of Early Outcome.

BACKGROUND: Surgery in grown-ups with congenital heart disease (GUCH) is characterized by complex anatomy, comorbidities, reoperations, and technical challenges. Although 30-day postoperative mortality is low, this measure might be insufficient to reflect adverse outcome monitoring. Our study aimed to establish whether prolonged intensive care unit (ICU) stay (≥7 days) and 6-month mortality were more clinically meaningful measures than 30-day mortality and to identify predictors of adverse outcome. METHODS: All consecutive GUCH patients from 1998 to 2015 were identified. Perioperative characteristics, diagnoses, and postoperative data were collected retrospectively. Predictors of 30-day and 6-month mortality and prolonged ICU stay were determined with logistic regression. Era effect was tested for quality assurances by dividing the cohort into 4 time intervals. RESULTS: Within 17 years, 1093 consecutive cardiac surgical procedures were identified in 1026 GUCH patients. During the study period, 30-day mortality improved significantly, with an overall 30-day mortality of 1.5%; 6-month mortality and prolonged ICU stay were 2.4% and 6.7%, respectively. Despite a decreased number of preoperative patients in New York Heart Association Functional Classification III or higher, prolonged ICU stay increased over the eras. Predictors of adverse outcome were New York Heart Association class III or higher, preoperative renal failure, disease of great complexity, preoperative ventilator support, cardiopulmonary bypass time, and concomitant procedures. CONCLUSIONS: In the current era of low 30-day mortality, extended 6-month mortality and prolonged ICU stay reporting may be more realistic measures of adverse outcomes for counseling GUCH patients at risk.

Clinical issues and outcomes in adults following repair of tetralogy of fallot.

OPINION STATEMENT: Advances in diagnosis, perioperative and surgical management of neonates born with Tetralogy of Fallot (TOF) have meant that affected individuals can now expect to survive to adulthood. However, this success is counterbalanced by the development of late morbidity in adulthood affecting survival and functional capacity. This review article addresses some of the major sequelae of TOF repair and discusses the contemporary approaches to reduce morbidity and mortality in this population.

Early versus late functional outcome after successful percutaneous pulmonary valve implantation: are the acute effects of altered right ventricular loading all we can expect?

OBJECTIVES: The purpose of this study was to assess the potential of late positive functional remodeling after percutaneous pulmonary valve implantation (PPVI) in right ventricular outflow tract dysfunction. BACKGROUND: PPVI has been shown to impact acutely on biventricular function and exercise performance, but the potential for further late functional remodeling remains unknown. METHODS: Sixty-five patients with sustained hemodynamic effects of PPVI at 1 year were included. Patients were divided into 2 subgroups based on pre-procedural predominant pulmonary stenosis (PS) (n = 35) or predominant pulmonary regurgitation (PR) (n = 30). Data from magnetic resonance imaging and cardiopulmonary exercise testing were compared at 3 time points: before PPVI, within 1 month (early) and at 12 months (late) after PPVI. RESULTS: There was a significant decrease in right ventricle end-diastolic volume early after PPVI in both subgroups of patients. Right ventricle ejection fraction improved early only in the PS group (51 ± 11% vs. 58 ± 11% and 51 ± 12% vs. 50 ± 11%, p < 0.001 for PS, p = 0.13 for PR). Late after intervention, there were no further changes in magnetic resonance parameters in either group (right ventricle ejection fraction, 58 ± 11% in the PS group and 52 ± 11% in the PR group, p = 1.00 and p = 0.13, respectively). In the PS group at cardiopulmonary exercise testing, there was a significant improvement in peak oxygen uptake early (24 ± 8 ml/kg/min vs. 27 ± 9 ml/kg/min, p = 0.008), with no further significant change late (27 ± 9 ml/kg/min, p = 1.00). In the PR group, no significant changes in peak oxygen uptake from early to late could be demonstrated (25 ± 8 ml/kg/min vs. 25 ± 8 ml/kg/min vs. 26 ± 9 ml/kg/min, p = 0.48). CONCLUSIONS: In patients with a sustained hemodynamic result 1 year after PPVI, a prolonged phase of maintained cardiac function is observed. However, there is no evidence for further positive functional remodeling beyond the acute effects of PPVI.

Electrical remodeling following percutaneous pulmonary valve implantation.

Sudden cardiac death in congenital heart disease is related to increased right ventricular end-diastolic volume (RVEDV), abnormalities of QRS duration, and QRS, JT, and QT dispersions. Surgical pulmonary valve replacement and percutaneous pulmonary valve implantation (PPVI) decrease RVEDV, but the effects of PPVI on surface electrocardiographic parameters are unknown. PPVI represents a pure model of RV mechanical and electrophysiologic changes after replacement. This prospective study sought to determine the effects of PPVI on surface electrocardiographic parameters: Ninety-nine PPVI procedures in patients with congenital heart disease (23.1 ± 10 years of age) were studied before, after, and 1 year after PPVI with serial electrocardiograms and echocardiogram/magnetic resonance images. Forty-three percent had pulmonary stenosis, 27% pulmonary regurgitation (PR), and 29% mixed lesions. In those with predominantly PR (n = 26), QRS duration decreased significantly (135 ± 27 to 128 ± 29 ms, p = 0.007). However, in the total cohort no significant change in QRS duration at 1 year was observed (137 ± 29 to 134 ± 29 ms). Corrected QT interval and QRS, QT, and JT dispersions significantly decreased at 1 year (p ≤0.001). RVEDV correlated with preprocedure QRS duration (r = 0.34, p <0.002) but there was no correlation after PPVI. In conclusion, this is the first study reporting electrical remodeling after isolated PPVI and it confirms that decreases in QRS duration occur after PPVI in PR, as reported for equivalent surgical cohorts. Further, increased homogeneity of repolarization in combination with improved conduction may decrease arrhythmic events in congenital cardiac patients with pulmonary valvular disease.

The relationship of systemic right ventricular function to ECG parameters and NT-proBNP levels in adults with transposition of the great arteries late after Senning or Mustard surgery.

AIMS: Heart failure is common late after Senning or Mustard palliation of transposition of the great arteries (TGA). Although cardiac magnetic resonance (CMR) is the gold standard for evaluating systemic right ventricular performance, additional information regarding heart failure status might be gleaned from the surface ECG and circulating N-terminal pro-brain natriuretic peptide (NT-proBNP) levels. The interrelationships between these heart failure markers were examined in adults late after Mustard and Senning surgery. METHODS: Thirty-five consecutive adults with Senning or Mustard repair of TGA attending a dedicated congenital heart failure clinic were studied. Assessment included symptom assessment, venous blood sampling for measurement of circulating NT-proBNP levels, surface 12-lead ECG and CMR for the assessment of right ventricular systolic function and determination of indexed right ventricular volumes. RESULTS: Mean age was 29 ± 6.5 years, 54% had undergone Mustard surgery. Compared with those with uncomplicated surgery, patients with complex surgical history had higher NT-proBNP levels (55 ± 26 vs 20 ± 35 pmol/l; p=0.002) and longer QRS duration (116 ± 28 ms vs 89 ± 11 ms; p=0.0004) while showing no difference in New York Heart Association class and right ventricular function. There was a significant relationship between diastolic and systolic right ventricular volumes and both NT-proBNP levels (r=0.43, p=0.01; r=0.53, p=0.001, respectively) and QRS duration (r=0.47, p=0.004; r=0.53, p=0.001, respectively). CONCLUSIONS: Circulating NT-proBNP levels and several surface ECG parameters constitute safe, cost-effective and widely available surrogate markers of systemic right ventricular function and provide additional information on heart failure status. Both measures hold promise as prognostic markers and their association with long-term outcome should be determined.

Functional outcomes after the Ross (pulmonary autograft) procedure assessed with magnetic resonance imaging and cardiopulmonary exercise testing.

OBJECTIVE: To assess autograft, homograft and ventricular function, as well as exercise capacity, in adult patients who have undergone the Ross procedure. SETTING: Single centre paediatric and adult congenital heart disease unit. Patients 45 subjects (24.6 years, range 16.9-52.2 years) who underwent the Ross procedure between 1994 and 2006 (8.1 years after the Ross operation, range 2.0-14.0 years). Interventions Cardiovascular magnetic resonance imaging, echocardiography and cardiopulmonary exercise testing. MAIN OUTCOME MEASURES: Autograft and homograft stenosis, and regurgitation. Autograft size. Biventricular function, scar volume and exercise capacity. RESULTS: Mean autograft regurgitation was 6.8%+/-8.3% (trivial regurgitation) and diameter was 40.0+/-7.0 mm. Mean homograft velocity was 2.4+/-0.6 m/s (mild-moderate stenosis) and regurgitation was 6.1%+/-8.3% (trivial regurgitation). Biventricular systolic function was normal (LV EF 63.1+/-6.4% and RV EF 60.1%+/-7.6%). In 38% of cases there was evidence of LV scar, mostly noted within the inter-ventricular septum. The mean exercise capacity achieved was 87%+/-22% of predicted. There was no correlation between exercise capacity and ventricular function or scar. CONCLUSION: This study demonstrates minor autograft and homograft dysfunction in the majority of patients after the Ross procedure, associated with good ventricular function and exercise capacity. In addition, minor scar was present in a third of patients with no functional consequences.

Late magnetic resonance surveillance of repaired coarctation of the aorta.

OBJECTIVE: Coarctation of the aorta has often been described as a simple form of congenital heart disease. However, rates of re-coarctation reported in the literature vary from 7% to 60%. Re-coarctation of the aorta may lead to worsening systemic hypertension, coronary artery disease and/or congestive cardiac failure. We aimed to describe the rates of re-coarctation in subjects who had undergone early coarctation repair (<2 years of age) and referred for clinically indicated or routine magnetic resonance (MR) surveillance. METHODS: We retrospectively identified 50 consecutive subjects (20.2+/-6.9 years post-repair) imaged between 2004 and 2008. Patient characteristics, rates of re-coarctation and LV/aortic dimensions were examined. RESULTS: Forty percent of subjects had bicuspid aortic valves (BAV). There were 40 cases of end-to-end repair and 10 cases of subclavian flap repair. Re-intervention with balloon angioplasty or repeat surgery had been performed in 32% of subjects. The MRI referrals were clinically indicated in 34% and routine in 66% of patients. Re-coarctation was considered moderate or severe in 34%, mild in 34% and no re-coarctation was identified in 32% of patients. There was no significant difference in the number of cases of re-coarctation identified in the clinically indicated versus routine referrals for MR imaging (p=0.20). There were no cases of aortic dissection or aneurysm formation identified amongst the subjects. The mean indexed left ventricular mass and ejection fraction was 72+/-16g/m(2) and 66+/-6%, respectively. Amongst those subjects with BAV there were larger aortic sinus (30+/-1mm vs 27+/-1mm, p=0.03) and ascending aortic (27+/-1mm vs 23+/-1mm, p=0.01) dimensions when compared to subjects with morphologically tricuspid aortic valves. CONCLUSIONS: We demonstrate that many years after early repair of coarctation of the aorta, MR surveillance detects significant rates of re-coarctation. These findings were independent of whether or not there was a clinical indication for imaging. Those patients with BAV disease had larger ascending aortic dimensions and may require more frequent non-invasive surveillance.

Percutaneous pulmonary valve implantation: impact of evolving technology and learning curve on clinical outcome.

BACKGROUND: Percutaneous pulmonary valve implantation was introduced in the year 2000 as a nonsurgical treatment for patients with right ventricular outflow tract dysfunction. METHODS AND RESULTS: Between September 2000 and February 2007, 155 patients with stenosis and/or regurgitation underwent percutaneous pulmonary valve implantation. This led to significant reduction in right ventricular systolic pressure (from 63+/-18 to 45+/-13 mm Hg, P<0.001) and right ventricular outflow tract gradient (from 37+/-20 to 17+/-10 mm Hg, P<0.001). Follow-up ranged from 0 to 83.7 months (median 28.4 months). Freedom from reoperation was 93% (+/-2%), 86% (+/-3%), 84% (+/-4%), and 70% (+/-13%) at 10, 30, 50, and 70 months, respectively. Freedom from transcatheter reintervention was 95% (+/-2%), 87% (+/-3%), 73% (+/-6%), and 73% (+/-6%) at 10, 30, 50, and 70 months, respectively. Survival at 83 months was 96.9%. On time-dependent analysis, the first series of 50 patients (log-rank test P<0.001) and patients with a residual gradient >25 mm Hg (log-rank test P=0.01) were associated with a higher risk of reoperations. CONCLUSIONS: Percutaneous pulmonary valve implantation resulted in the ability to avoid surgical right ventricular outflow tract revision in the majority of cases. This procedure might reduce the number of operations needed over the total lifetime of patients with right ventricle-to-pulmonary artery conduits.

Percutaneous pulmonary valve-in-valve implantation: a successful treatment concept for early device failure.

AIMS: Percutaneous pulmonary valve implantation (PPVI) is now an accepted treatment strategy for right ventricular (RV) outflow tract (RVOT) dysfunction in many European Heart Centres. We analysed the efficacy of repeat PPVI as a treatment modality for early device failure. METHODS AND RESULTS: Twenty patients underwent repeat PPVI for RVOT obstruction because of early device failure ('Hammock effect', 'Hammock-like effect', stent fracture, residual stenosis). Repeat PPVI was feasible in all patients with no procedural complications. Following implantation of a second device, catheter-measured RVOT gradient and RV systolic pressure fell significantly (RVOT gradient: 46.1 +/- 3.9 to 18.1 +/- 2.4 mmHg, P < 0.001; RVSP: 70.9 +/- 4.8 to 46.1 +/- 2.6 mmHg, P < 0.001), in all but one patient (15 years, male, common arterial trunk, 11.5 mm homograft). During follow-up, four of 20 required re-intervention [third PPVI for stent fracture (n = 2), device explantation: external compression by the sternum (n = 1), endocarditis (n = 1)], and one of the 20 is awaiting surgical management. In the remainder, second PPVI resulted in a sustained improvement in haemodynamics with a mean follow-up of 10.9 +/- 3.0 months. In this series, the probability of freedom from re-intervention at 2 years was higher after second PPVI when compared with the index procedure (89.4 vs. 20.0%, P < 0.001). CONCLUSION: Repeat PPVI is an effective treatment for early device failure in defined conditions and leads to improved freedom from re-intervention.

Comparison of noncontact and electroanatomic mapping to identify scar and arrhythmia late after the Fontan procedure.

BACKGROUND: The right atrium late after the Fontan procedure is characterized by multiple complex arrhythmia circuits. We performed simultaneous electroanatomic and noncontact mapping to assess the accuracy of both systems to identify scar and arrhythmia. METHODS AND RESULTS: Mapping was performed in 26 patients aged 26.8+/-8.9 years, 18.7+/-4.4 years after Fontan surgery. The area and site of abnormal endocardium defined by electroanatomic mapping (bipolar contact electrogram <0.5 mV) were compared with those defined by noncontact mapping during sinus rhythm and by dynamic substrate mapping. Contact and reconstructed unipolar electrograms at a known distance from the multielectrode array, recorded by the noncontact system simultaneously at 452 endocardial sites, were compared for morphological cross correlation, timing difference, and amplitude. Mapping of arrhythmias was performed with both systems when possible. The median patient abnormal endocardium as defined by electroanatomic mapping covered 38.0% (range 16.7% to 97.8%) of the right atrial surface area, as opposed to 60.9% (range 21.3% to 98.5%) defined by noncontact mapping during sinus rhythm and 11.9% (range 0.4% to 67.3%) by dynamic substrate mapping. A significant decrease in electrogram cross correlation (P=0.003), timing (P=0.012), and amplitude (P=0.003) of reconstructed electrograms, but not of contact electrograms (P=0.742), was seen at endocardial sites >40 mm from the multielectrode array. Successful arrhythmia mapping by electroanatomic versus noncontact mapping was superior in 15 patients (58%), the same in 6 (23%), and inferior in 5 (19%; P=0.044). CONCLUSIONS: Electroanatomic mapping is the superior modality for arrhythmia mapping late after the Fontan procedure. Noncontact mapping is limited by a significant reduction in reconstructed electrogram correlation, timing, and amplitude >40 mm from the multielectrode array and cannot accurately define areas of scar and low-voltage endocardium.

The care of adult patients with congenital heart defects: a new challenge.

The aim of the present article was to point out some of the more common challenges and needs for adult patients with congenital heart defects. We may reasonably calculate a population approximating 80,000 to 100,000 patients in Italy. The profile of this patient population will change over the next few decades. Not all congenital heart defects require the same level of expertise; for this reason an integrated national service is required. Ideally, specialist units should be established in appropriate geographic areas; complex patients need to be grouped according to the expertise, experience and management they require. Less specialized regional centers and outpatient clinics in interconnected districts with GUCH units should be created. Specialist units should accept responsibility for educating the profession, training the specialists, and sharing particular skills between each other. The debate on this subject is far from over; we wish to contribute to and stimulate the discussion.

Systemic ventricular function in patients with transposition of the great arteries after atrial repair: a tissue Doppler and conductance catheter study.

OBJECTIVES: The aim of this study was to assess the utility of tissue Doppler echocardiography in the setting of repaired transposition of the great arteries when the right ventricle (RV) functions as the systemic ventricle. BACKGROUND: Myocardial acceleration during isovolumic contraction, "isovolumic myocardial acceleration" (IVA), has been validated as a sensitive non-invasive method of assessing RV contractility. Although traditional indexes may be less valid for the abnormal RV, the relative insensitivity of IVA to an abnormal load makes it a potentially powerful clinical tool for the assessment of RV disease. METHODS: We examined 55 controls and 80 patients (mean age 22 years) with transposition, who had undergone atrial repair at age 8 (0.3 to 72) months. A subgroup of 12 underwent cardiac catheterization. The RV systolic function was derived by analysis of pressure-volume relationships and IVA both at rest and during dobutamine stress. In all 80, myocardial velocities were sampled in the RV free wall. RESULTS: During dobutamine (10 microg/kg/min for 10 min), the increase of IVA mirrored the increase in end-systolic elastance (r = 0.69, p < 0.02). In the group as a whole, IVA was reduced compared with the subpulmonary RV and the systemic left ventricle of controls. There was abnormal wall motion in 44 patients, which was associated with reduced IVA. Diastolic myocardial velocities were also abnormal but unrelated to the presence of wall motion abnormalities. CONCLUSIONS: The IVA can accurately assess changes in RV contractile function in patients with an RV as the systemic ventricle. Global long-axis RV function is reduced in patients with transposition, and this is associated with abnormal regional function.