Predicting Survival in Repaired Tetralogy of Fallot: A Lesion-Specific and Personalized Approach.

OBJECTIVES: This study sought to identify patients with repaired tetralogy of Fallot (rTOF) at high risk of death and malignant ventricular arrhythmia (VA). BACKGROUND: To date there is no robust risk stratification scheme to predict outcomes in adults with rTOF. METHODS: Consecutive patients were prospectively recruited for late gadolinium enhancement (LGE) cardiovascular magnetic resonance (CMR) to define right and left ventricular (RV, LV) fibrosis in addition to proven risk markers. RESULTS: The primary endpoint was all-cause mortality. Of the 550 patients (median age 32 years, 56% male), 27 died (mean follow-up 6.4 ± 5.8; total 3,512 years). Mortality was independently predicted by RVLGE extent, presence of LVLGE, RV ejection fraction ≤47%, LV ejection fraction ≤55%, B-type natriuretic peptide ≥127 ng/L, peak exercise oxygen uptake (V02) ≤17 mL/kg/min, prior sustained atrial arrhythmia, and age ≥50 years. The weighted scores for each of the preceding independent predictors differentiated a high-risk subgroup of patients with a 4.4%, annual risk of mortality (area under the curve [AUC]: 0.87; P < 0.001). The secondary endpoint (VA), a composite of life-threatening sustained ventricular tachycardia/resuscitated ventricular fibrillation/sudden cardiac death occurred in 29. Weighted scores that included several predictors of mortality and RV outflow tract akinetic length ≥55 mm and RV systolic pressure ≥47 mm Hg identified high-risk patients with a 3.7% annual risk of VA (AUC: 0.79; P < 0.001) RVLGE was heavily weighted in both risk scores caused by its strong relative prognostic value. CONCLUSIONS: We present a score integrating multiple appropriately weighted risk factors to identify the subgroup of patients with rTOF who are at high annual risk of death who may benefit from targeted therapy.

Long-term mortality and cardiovascular burden for adult survivors of coarctation of the aorta.

OBJECTIVE: To examine the contemporary long-term outcome after coarctation repair. METHODS: This is a retrospective cohort study of 834 patients aged ≥16 years who underwent coarctation repair under single-centre follow-up. Repair was performed at a median age of 3 years (lower-upper quartile: 1 month to 15 years) by surgery in 83% (690/834) and angioplasty/stenting in 17% (144/834). Survival was compared with an age- and gender matched normal population. Other outcomes included arch reintervention, aortic valve intervention, ascending aortic intervention, and residual/re-coarctation and resting hypertension at latest follow-up. RESULTS: After a median follow-up of 27 years (lower-upper quartile: 18-36), there were 38 late deaths (5%, 38/834). Overall survival was 99%, 88% and 65% at 30, 50 and 70 years of age, respectively, significantly reduced compared with a matched normal population (standardised mortality ratio: 3.20, log-rank: p<0.001). Thirty per cent (246/834) required ≥1 arch reintervention, 13% (111/834) an aortic valve intervention and 5% (43/834) an ascending aortic intervention. Freedom from aortic valve and ascending aortic intervention was 83% and 92% at 50 years and 53% and 81% at 70 years of age, respectively. Residual/re-coarctation (gradient ≥25 mm Hg or repair site/diaphragm ratio ≤70%) at latest follow-up was present in 60% (282/474) and resting hypertension in 57% (379/661). CONCLUSIONS: Long-term survival in contemporary adult survivors of coarctation repair is significantly lower than a matched normal population with accelerated decline after the third decade. Nearly 60% of patients eventually develop hypertension, whereas approximately 50% require further invasive cardiovascular treatment by 50 years of age. Our risk-stratifying data may enable personalised follow-up strategies for this common congenital heart condition.

Surgical and percutaneous pulmonary valve replacement in England over the past two decades.

OBJECTIVE: Pulmonary valve replacement (PVR) is often required in patients with congenital heart disease. We aimed to describe temporal trends in PVR in a nationwide English cohort between 1997 and 2014, survival and the need for re-PVR. METHODS: Patients were identified in the Hospital Episode Statistics Database. Survival data were retrieved from the UK Office for National Statistics. RESULTS: A total of 2733 patients underwent PVR (2845 procedures) over the study period. Median age at first procedure increased from 20.1 years in 1997-2005 to 24.7 years in 2006-2014. The annual number of PVRs increased from 23 in 1997 to 251 in 2014. Homografts were the most common choice in the early years, but the use of xenografts increased after 2005. During a median follow-up of 5.8 years, 176 patients died and 108 required redo PVR. Early (30 day) survival was 98% for all PVRs and was similar for all types of prostheses but longer-term mortality dropped to 92% at 10 years and 90% at 15 years. Age >16 years and percutaneous PVR were risk factors for death. The cumulative incidence for re-PVR at 10 years was 8% for all PVRs and 11% at 15 years. Risk factors for re-PVR were complex diagnosis, male gender and black ethnicity. CONCLUSION: There was a significant increase in the number of PVRs performed in England over the last two decades and a significant change in the type of prosthesis employed. While early mortality was low across the board, longer-term mortality was not negligible in this young population.

Modern management of adult coarctation: transcatheter and surgical options.

Coarctation of the aorta (CoA), a juxtaductal obstructive lesion in the descending aorta and commonly associated with hypoplasia of the aortic arch occurs in 5-8% of patients with congenital heart disease. Since the initial surgical corrections in the 1950, surgical and transcatheter options have constantly evolved. Nowadays, transcatheter options are widely accepted as the initial treatment of choice in adults presenting with native or recurrent CoA. Surgical techniques are mainly reserved for patients with complex aortic arch anatomy such as extended arch hypoplasia or stenosis or para-CoA aneurysm formation. Extended aneurysms can be covered by conformable stents but stent implantation may require preparative vascular surgery. Complex re-CoA my best be treated by an ascending to descending bypass conduit. The following review aims to describe current endovascular and surgical practice pointing out modern developments and their limitations.

Clinical outcomes of surgical pulmonary valve replacement after repair of tetralogy of Fallot and potential prognostic value of preoperative cardiopulmonary exercise testing.

BACKGROUND: Indications for surgical pulmonary valve replacement (PVR) after repair of tetralogy of Fallot have recently been broadened to include asymptomatic patients. METHODS AND RESULTS: The outcomes of PVR in adults after repair of tetralogy of Fallot at a single tertiary center were retrospectively studied. Preoperative cardiopulmonary exercise testing was included. Mortality was the primary outcome measure. In total, 221 PVRs were performed in 220 patients (130 male patients; median age, 32 years; range, 16-64 years). Homografts were used in 117 patients, xenografts in 103 patients, and a mechanical valve in 1 patient. Early (30-day) mortality was 2%. Overall survival was 97% at 1 year, 96% at 3 years, and 92% at 10 years. Survival after PVR in the later era (2005-2010; n=156) was significantly better compared with survival in the earlier era (1993-2004; n=65; 99% versus 94% at 1 year and 98% versus 92% at 3 years, respectively; P=0.019). Earlier era patients were more symptomatic preoperatively (P=0.036) with a lower preoperative peak oxygen consumption (peak Vo2; P<0.001). Freedom from redo surgical or transcatheter PVR was 98% at 5 years and 96% at 10 years for the whole cohort. Peak Vo2, E/CO2 slope (ratio of minute ventilation to carbon dioxide production), and heart rate reserve during cardiopulmonary exercise testing predicted risk of early mortality when analyzed with logistic regression analysis; peak Vo2 emerged as the strongest predictor on multivariable analysis (odds ratio, 0.65 per 1 mL·kg⁻¹·min⁻¹; P=0.041). CONCLUSIONS: PVR after repair of tetralogy of Fallot has a low and improving mortality, with a low need for reintervention. Preoperative cardiopulmonary exercise testing predicts surgical outcome and should therefore be included in the routine assessment of these patients.

Outcome in adult patients after arterial switch operation for transposition of the great arteries.

BACKGROUND: The arterial switch operation (ASO) is currently the treatment of choice in neonates with transposition of the great arteries (TGA). The outcome in childhood is encouraging but only limited data for long-term outcome into adulthood exist. METHODS AND RESULTS: We studied 145 adult patients (age>16, median 25 years) with ASO followed at our institution. Three patients died in adulthood (mortality 2.4/1000-patient-years). Most patients were asymptomatic and had normal left ventricular function. Coronary lesions requiring interventions were rare (3 patients) and in most patients related to previous surgery. There were no acute coronary syndromes. Aortic root dilatation was frequent (56% patients) but rarely significant (>45 mm in 3 patients, maximal-diameter 49 mm) and appeared not to be progressive. There were no acute aortic events and no patient required elective aortic root surgery. Progressive neo-aortic-valve dysfunction was not observed in our cohort and only 1 patient required neo-aortic-valve replacement. Many patients (42.1%), however, had significant residual lesions or required reintervention in adulthood. Right ventricular outflow tract lesions or dysfunction of the neo-pulmonary-valve were frequent and 8 patients (6%) required neo-pulmonary-valve replacement. Cardiac interventions during childhood (OR 3.0, 95% CI 1.7-5.4, P<0.0001) were strong predictors of outcome (cardiac intervention/significant residual lesion/death) in adulthood. CONCLUSIONS: Adult patients with previous ASO remain free of acute coronary or aortic complications and have low mortality. However, a large proportion of patients require re-interventions or present with significant right sided lesions. Life-long cardiac follow-up is, therefore, warranted. Periodic noninvasive surveillance for coronary complications appears to be safe in adult ASO patients.

Adult congenital heart disease: a 2008 overview.

INTRODUCTION: During the past decades, health care of patients born with congenital heart disease (CHD) has improved substantially, leading to a growing population of adult survivors. SOURCE OF DATA: Using the recently published and relevant data on adult CHD (ACHD), we reviewed the most common congenital heart defects and discussed important related issues. AREAS OF AGREEMENT: Adults with CHD most often require specialized medical or surgical care in a tertiary centre. However, this population also need local follow-up; general practitioners and other specialists therefore have to face the complexity of their disease. AREAS OF CONTROVERSIES: Management of pregnancy, non-cardiac surgery, arrhythmias and endocarditis prophylaxis may be challenging in patients with CHD and should be adapted to their condition. GROWING POINTS: The present article summarizes key clinical information on ACHD for the benefit of physicians who are not specialized in this field. Areas timely for developing research Research efforts and education strategies are greatly needed in order to optimize the care of patients with ACHD.

Congenital partial absence of the left pericardium associated with tricuspid regurgitation.

We report the case of a 47-year-old man who presented with several episodes of left precordial pain, one of which had been severe, but was unrelated to exertion or posture. Transthoracic echocardiography and cardiovascular magnetic resonance showed evidence of congenital partial absence of the left pericardium and severe tricuspid regurgitation. Both diagnoses were confirmed at surgery when the pericardial defect was repaired and the tricuspid valve was replaced at the same operation. He went on to make a good recovery.

Surgery for complex coarctation of the aorta.

The application of improved surgical techniques to correct coarctation in the neonate and infant may in the longterm reduce the incidence of recoarctation in the adult. In addition, in many cases, catheter-based intervention offers an alternative to reoperation and the role of catheter-based intervention is likely to be extended with the introduction of improved technology. However, surgery is likely to be required in coarctation and recoarctation in the adult in some cases, particularly those with hypoplasia of the aortic arch, aneurysms of the ascending aorta and those with intracardiac pathology. Operation for primary coarctation in the adult can be performed through a left thoracotomy without significant postoperative morbidity. However, this is not the case with recoarctation where reoperation through a left thoracotomy has resulted in a high incidence of postoperative complications including residual coarctation, false aneurysm and recurrent laryngeal nerve palsy. Repair through a median sternotomy offers an alternative surgical approach to recoarctation which avoids these complications and allows concomitant procedures for problems associated with arteriopathy, aortic valve disease and other associated intracardiac anomalies. The approach to coarctation and recoarctation in the adult should be tailored to individual patients and made after careful discussion with interventional cardiologists.

Timing and type of surgery for severe pulmonary regurgitation after repair of tetralogy of Fallot.

Repaired tetralogy of Fallot (rTOF) has an excellent long-term prognosis; however, survival is somewhat less than normal. Of all the residual lesions and sequellae after rTOF, pulmonary regurgitation (PR) is the most important, correlating with right ventricular (RV) size, exercise intolerance and serious ventricular arrhythmias. Pulmonary valve replacement (PVR) has beneficial effects on RV size and function, provided it is performed early, before irreversible RV dysfunction ensues. Moreover, PVR is associated with an improvement in patients' symptoms and exercise tolerance and combined with arrhythmia surgery (cryoablation) it leads to a dramatic decrease in the incidence of fatal ventricular arrhythmias. Associated lesions, especially branch pulmonary artery stenosis, which aggravates PR, and tricuspid regurgitation, which further impacts on RV size and function, need addressing. Large right ventricular outflow (RVOT) akinetic and aneurysmal regions are frequent and further compromise RV function; therefore, resection during PVR should be attempted. Despite excellent mid-term results, homografts and xenografts, usually used for RVOT reconstruction, suffer late dysfunction and failure, committing patients and surgeons to further operations. Therefore, the decision to operate should be based on the balance between progressive RV dilatation, exercise intolerance, symptoms, arrhythmias and the fact that further reoperations will be needed. Research on the ideal valve for RVOT reconstruction is ongoing. Prospective follow-up of patients with rTOF with exercise testing and assessment of RV size and function, preferably with magnetic resonance, will define better the natural history of the disease and will probably provide firm guidelines for PVR timing especially in asymptomatic patients.

Re-do aortic valve replacement: does a previous homograft influence the operative outcome?

BACKGROUND AND AIM OF THE STUDY: Late reoperation for failed aortic homograft is widely regarded as a high-risk procedure. A review is presented of the authors' experience of redo-aortic valve replacement (re-do AVR) examining factors which affect, and whether a previous aortic homograft replacement influences, operative outcome. METHODS: A retrospective review was conducted of consecutive re-do AVR performed at the authors' institution between 1998 and 2002. RESULTS: During the study period, 178 patients (125 males, 53 females; mean age 52.4 years; range: 16-85 years) underwent re-do AVR. The group included first-time (72%), second-time (20%), and more than third-time re-do AVR (8%). Forty-six patients (26%) received a homograft (group I), and 132 (74%) a stented biological/mechanical valve (group II). The two groups were matched for baseline clinical characteristics and operative variables. The type of explanted valve, and preoperative and operative variables, were analyzed using univariate and multivariate models. Primary outcome was defined as 30-day mortality, and secondary outcome as postoperative complications. The overall 30-day mortality was 12.3%, but was much lower (4.5%) for elective isolated and multiple re-do AVR. Univariate analysis showed significant predictors of 30-day mortality to be: age >65 years (p = 0.02); renal dysfunction (p = 0.005); preoperative unstable status (p = 0.03); preoperative NYHA class III/IV dyspnea (p = 0.02); non-elective operation (p = 0.01); preoperative arrhythmia (p = 0.005); history of chronic obstructive pulmonary disease (COPD) (p = 0.002); preoperative cardiogenic shock (p = 0.03); impaired left ventricular ejection fraction (LVEF) <50% (p = 0.04); and other valvular procedure(s) performed simultaneously (p = 0.01). In a multivariate analysis, the only significant predictors of 30-day mortality were impaired LVEF (p = 0.03) and a history of COPD (p = 0.007). Group I patients had a significantly shorter mean hospital stay (10.2+/-5.9 versus 14.1+/-12.5 days; p = 0.009), but there were no significant differences between groups in terms of postoperative complications. CONCLUSION: A previous aortic homograft replacement was not associated with an increased operative risk at the time of re-do AVR. A history was COPD was an important predictor of 30-day mortality, and this finding requires further investigation.

The left atrioventricular valve in partial atrioventricular septal defect: management strategy and surgical outcome.

OBJECTIVE: To test the hypothesis that in patients with a partial atrioventricular septal defect (PAVSD) and a competent left atrioventricular valve (LAVV), sutures should be placed across the line of apposition of the superior and inferior bridging leaflets, septal commissure (SC), to prevent the development of regurgitation. Outcome of surgery and risk factors for the need for LAVV reoperation of patients with mild or no LAVV regurgitation (LAVVR) were evaluated. BACKGROUND: Controversy over management of the LAVV in PAVSD. METHOD: One hundred and forty seven children with PAVSD underwent surgical repair at the Royal Brompton Hospital between January 1983 and December 1999. Of this group, 21 (16.7%) had LAVVR of sufficient severity to require surgical intervention and were therefore excluded from analysis. The median age and weight at repair of those with mild or no LAVVR was 4.1 years and 15.4 kg. One hundred and eight had normal chromosomes, 13 Down syndrome and five other syndromes. The interatrial communication was closed using a pericardial patch in 62.7% and with synthetic material in the remainder. Intraoperative testing of LAVV competence was undertaken using saline injection into the left ventricle. In 80.9%, sutures were placed across the line of apposition of the left sided superior and inferior bridging leaflets partially to close the SC (sometimes incorrectly named the mitral valve cleft). RESULT: The overall hospital mortality was 3.2% (95% confidence interval (CI) 1, 8.4%), which did not differ statistically in the last 20 years. No specific risk factors for early death were identified. Eleven patients (8.7%, 95% CI 4.7, 15.4%) required reoperation, 10 for LAVV repair and 1 resection of subaortic stenosis. Univariate analysis of risk factors for LAVV reoperation were low weight, relatively small size LAVV, the presence of a small preoperative interventricular interchordal communication and duration of ventilation. Ten (9.8%) of 102 patients in whom SC was sutured required LAVV reoperation but none for 24 in whom the commissure was left alone. CONCLUSION: The hypothesis that in the absence of preoperative LAVVR it is necessary to place sutures in the SC has not been proven. We consider that in addition to preoperative cross sectional echocardiographic assessment of LAVVR intraoperative evaluation of LAVV function allows discrimination between those valves where sutures to the septal commissure are necessary and those where the valve can be left undisturbed.

Complete atrioventricular septal defect, Down syndrome, and surgical outcome: risk factors.

BACKGROUND: This study was conducted to evaluate surgical outcome and to identify risk factors for hospital mortality and reoperation after repair of complete atrioventricular septal defect. METHODS: A total of 147 consecutive children underwent repair between January 1986 and December 1998. Of those, 106 had Down syndrome, 37% had normal chromosomes, and 4 had other syndromes; 108 underwent primary repair, 19 had prior pulmonary artery banding, and 20 had additional tetralogy of Fallot. The median weight at primary repair was 4.5 kg. A two-patch technique was used in 88%. RESULTS: The 30-day mortality was 15% (70% confidence interval [CI] 12% to 19%). A double orifice atrioventricular valve was found to be a significant risk factor (p = 0.002), with 6 of 11 patients dying. If double orifice atrioventricular valve patients are excluded, the mortality rate falls to 12% (70% CI 9% to 15%). No difference in mortality was found between Down syndrome and chromosomally normal children but the latter more commonly required reoperation. Chromosomally normal children frequently have a dysplastic common atrioventricular valve (24% versus 3% in Down children, p < 0.001). In a multivariate Cox model including both variables, the presence of a dysplastic atrioventricular valve was a significant risk factor for reoperation. After controlling for the presence of a dysplastic atrioventricular valve, Down syndrome retained a significant protective effect but the upper limit of the confidence interval was close to 1. CONCLUSIONS: The presence of a double orifice atrioventricular valve emerged as an unforeseen risk factor for death.