Gruschen Veldtman

Late risk of outcomes for adults with repaired tetralogy of Fallot from an inception cohort spanning four decades

BACKGROUND Adult survivors with tetralogy of Fallot constitute a growing population with congenital heart disease. We investigated an inception cohort who underwent surgical repair. We aimed to characterize late hazard or risk for death, and determine the time-related risk of late re-operation and pulmonary valve replacement (PVR). METHODS All children (n=1181) with tetralogy of Fallot born before 1984 who underwent surgical repair at our institution were included. Follow-up (median 20 years after repair) was obtained from 2003 to 2006 via chart review, clinic consultation and telephone interview. Outcomes were analyzed using parametric and competing risks techniques with bagging. RESULTS Corrective repair performed from 1960 to 1998 included transannular patch (n=370), right ventricular outflow tract patch (n=326), no patch (n=333) and right ventricular-pulmonary artery conduit (n=54). Overall, 85+/-1% survived to adulthood (age >18 years). Thirty years after repair, survival was 80+/-1%, instantaneous hazard or risk of death was 0.5+/-0.07% per year and half of survivors had undergone surgical re-operation. Surgical era of repair did not influence late risk of death. Therefore, with early surgical mortality <2% since 1985, 20-year survival has improved to 94+/-1%. If trends in late risks match those of the earliest repairs, 40-year survival will be approximately 90% for children repaired in the modern era. Pulmonary atresia variant (n=88) was associated with three-fold higher late risk of death than classic tetralogy of Fallot (n=1069). Presence of associated branch pulmonary artery stenosis or atrioventricular septal defects conferred a less optimal late prognosis. Risk of PVR was low, constant (0.8% per year) and independent of surgical era. Both pulmonary atresia and absent pulmonary valve (n=15) variants were associated with higher risk of late re-operation or PVR. Survival after re-operation or PVR (88+/-3% and 94+/-3% at 20 years, respectively) was excellent. CONCLUSIONS Surgical progress has not influenced late risks for death, re-operation or PVR in adults with repaired tetralogy of Fallot. Instead, reduction of early surgical mortality to <2% is responsible for excellent late survival >90% overall. The constant risk of PVR is low and independent of repair type. Baseline morphologic features are important determinants of late outcome.

Angiotensin-Converting Enzyme Inhibitors in Adults After the Mustard Procedure

Angiotensin-converting enzyme inhibitors had no significant effect on cardiopulmonary exercise function in 14 patients who had undergone a Mustard operation for transposition of the great arteries. In some patients aerobic capacity improved and maximum systolic blood pressure decreased.

Percutaneous Closure of Postinfarction Ventricular Septal Defect In-Hospital Outcomes and Long-Term Follow-Up of UK Experience

Background— Postinfarction ventricular septal defect carries a grim prognosis. Surgical repair offers reasonable outcomes in patients who survive a healing phase. Percutaneous device implantation represents a potentially attractive early alternative. Methods and Results— Postinfarction ventricular septal defect closure was attempted in 53 patients from 11 centers (1997–2012; aged 72±11 years; 42% female). Nineteen percent had previous surgical closure. Myocardial infarction was anterior (66%) or inferior (34%). Time from myocardial infarction to closure procedure was 13 (first and third quartiles, 5–54) days. Devices were successfully implanted in 89% of patients. Major immediate complications included procedural death (3.8%) and emergency cardiac surgery (7.5%). Immediate shunt reduction was graded as complete (23%), partial (62%), or none (15%). Median length of stay after the procedure was 5.0 (2.0–9.0) days. Fifty-eight percent survived to discharge and were followed up for 395 (63–1522) days, during which time 4 additional patients died (7.5%). Factors associated with death after postinfarction ventricular septal defect closure included the following: age (hazard ratio [HR]=1.04; P=0.039), female sex (HR=2.33; P=0.043), New York Heart Association class IV (HR=4.42; P=0.002), cardiogenic shock (HR=3.75; P=0.003), creatinine (HR=1.007; P=0.003), defect size (HR=1.09; P=0.026), inotropes (HR=4.18; P=0.005), and absence of revascularization therapy for presenting myocardial infarction (HR=3.28; P=0.009). Prior surgical closure (HR=0.12; P=0.040) and immediate shunt reduction (HR=0.49; P=0.037) were associated with survival. Conclusions— Percutaneous closure of postinfarction ventricular septal defect is a reasonably effective treatment for these extremely high-risk patients. Mortality remains high, but patients who survive to discharge do well in the longer term.Background— Postinfarction ventricular septal defect carries a grim prognosis. Surgical repair offers reasonable outcomes in patients who survive a healing phase. Percutaneous device implantation represents a potentially attractive early alternative. Methods and Results— Postinfarction ventricular septal defect closure was attempted in 53 patients from 11 centers (1997–2012; aged 72±11 years; 42% female). Nineteen percent had previous surgical closure. Myocardial infarction was anterior (66%) or inferior (34%). Time from myocardial infarction to closure procedure was 13 (first and third quartiles, 5–54) days. Devices were successfully implanted in 89% of patients. Major immediate complications included procedural death (3.8%) and emergency cardiac surgery (7.5%). Immediate shunt reduction was graded as complete (23%), partial (62%), or none (15%). Median length of stay after the procedure was 5.0 (2.0–9.0) days. Fifty-eight percent survived to discharge and were followed up for 395 (63–1522) days, during which time 4 additional patients died (7.5%). Factors associated with death after postinfarction ventricular septal defect closure included the following: age (hazard ratio [HR]=1.04; P =0.039), female sex (HR=2.33; P =0.043), New York Heart Association class IV (HR=4.42; P =0.002), cardiogenic shock (HR=3.75; P =0.003), creatinine (HR=1.007; P =0.003), defect size (HR=1.09; P =0.026), inotropes (HR=4.18; P =0.005), and absence of revascularization therapy for presenting myocardial infarction (HR=3.28; P =0.009). Prior surgical closure (HR=0.12; P =0.040) and immediate shunt reduction (HR=0.49; P =0.037) were associated with survival. Conclusions— Percutaneous closure of postinfarction ventricular septal defect is a reasonably effective treatment for these extremely high-risk patients. Mortality remains high, but patients who survive to discharge do well in the longer term. # CLINICAL PERSPECTIVE {#article-title-23}

Functional Health Status in Adult Survivors of Operative Repair of Tetralogy of Fallot

We aimed to determine late functional health status of the growing adult population with repaired tetralogy of Fallot (TOF). We studied all 840 patients with TOF born from 1927 through 1984 who survived to adulthood (> 18 years of age). Clinical follow-up was by chart review, telephone interview (n = 706), and echocardiographic reports (n = 339). Functional health status was assessed using Short Form-36 (SF-36) surveys (n = 396) indexed to normative data. Risk of reoperation was low (≈ 1%/year) but increased beyond age 40 years. At latest follow-up moderate or severe pulmonary regurgitation was common (54%) and right ventricular outflow tract stenosis presented in 1/3. Consequently, evidence of right ventricular dilatation and dysfunction and tricuspid regurgitation was typical. Left-sided abnormalities were also common: hypertrophy (p < 0.0001) and outflow tract dilation (p < 0.0001) with at least mild aortic regurgitation in > 50%. Cardiorespiratory symptoms were reported in 45% (palpitations 27%, dyspnea 21%, chest pain 17%). SF-36 scores were significantly below normal for 4 physical domains (p < 0.001). Decrements in physical functioning were associated particularly with older age at follow-up (p < 0.0001), associated syndromes/lesions, reoperations, ventricular dysfunction, tricuspid regurgitation, residual septal defects, and cardiorespiratory symptomatology. Echocardiographic abnormalities were more common in older patients (p < 0.0001). All 3 SF-36 domains specific to psychosocial well-being were normal. In conclusion, despite excellent survival prospects, physical compromise is common in adults with repaired TOF. Greater decrements in older patients may reflect late deterioration with advancing age or cohort effects related to historical management. Efforts to limit ventricular and outflow tract dysfunction may translate into improved late functional status.

Functional Health Status of Adults With Tetralogy of Fallot: Matched Comparison With Healthy Siblings

BACKGROUND Survival prospects for adults with repaired tetralogy of Fallot (TOF) are now excellent. Attention should therefore shift to assessing and improving functional health status and quality of life. We aimed to assess late functional health status of adults surviving TOF repair by matched comparison to their healthy siblings. METHODS All 1,693 TOF repairs performed at our institution between 1946 and 1990 were reviewed. A matched comparison was undertaken whereby presumed survivors and their healthy sibling were contacted and asked to complete the Ontario Health Survey 1990 and the 36-Item Short Form Health Survey (SF-36) questionnaire. RESULTS Both questionnaires were completed by 224 adult survivors and their sibling closest in age. Adults with repaired TOF had lower scores for self-perceived general health status (p < 0.001), were less likely to rate their health as good or excellent (p < 0.001), and had lower SF-36 scores for physical functioning and general health (p = 0.001) than their siblings. However, patients reported similar satisfaction with their lives, similar levels of social participation and support, and were as likely to be in long-term partnerships. Worse physical and mental health scores were associated with older age at surgery and at time of questionnaire completion and recent requirement for noncardiac medication. CONCLUSIONS Although reporting lower functional health status then their siblings, quality of life and life satisfaction for adults who underwent surgery for TOF during childhood is comparable to that of their siblings without heart defects. Follow-up of younger adults is required to understand current health outcomes attributable to improvements in the management of TOF.

Creation and Enlargement of Atrial Defects in Congenital Heart Disease

Transcatheter creation and enlargement of interatrial defects (IAD) may improve hemodynamics; however, procedural outcomes have not been well defined. Hospital records were reviewed for children who underwent percutaneous procedures to create and enlarge an IAD and were grouped as follows: (1) right and (2) left heart obstructive lesions, (3) left atrial (LA) decompression during left heart assist, (4) failing Fontan circulation, and (5) miscellaneous. Forty-five children (mean age, 3.4 ± 4.7 years; 30 (67%) male) were identified. In group 1 (n = 6), all achieved endpoints of right atrial (RA) decompression (n = 2), improved left ventricular filling (n = 3), or improved arterial saturations (n = 1). In group 2 (n =18), mean LA pressure decreased (21 ± 6 to 13 ± 5 mmHg, p < 0.001) and arterial saturations increased (61 ± 13% to 78 ± 11%, p < 0.001). All except 2 patients achieved definitive repair, further palliation (n = 9), or heart transplantation (HTX) (n = 7). In group 3 (n = 5), the LA was decompressed (21 to 13 mmHg, p = 0.03) in all, and all except 1 patient survived to HTX (n = 2) or full recovery (n = 2). In group 4 (n = 11), of 7 patients with a low cardiac output syndrome after surgery, despite improved atrial shunting, 3 died and 1 required a HTX. In group 5 (n=5), RA decompression (n = 1) or improved arterial saturation (n = 4) was achieved in all. Overall, 5-year HTX free survival was 75%. Mechanical ventilation before the procedure (p < 0.001), the need for a blade septostomy (p = 0.002), and higher LA pressures after the procedure (p = 0.04) independently predicted mortality or the requirement for HTX. Transcatheter optimization of an atrial communication can help optimize treatment strategies and has a low procedural risk.

Radiofrequency perforation and conventional needle percutaneous transseptal left heart access: pathological features.

Perforating radiofrequency (PRF) energy has been used to obtain percutaneous transseptal left heart access. Contrary to ablative radiofrequency (RF), myocardial tissue responses to PRF thermal injury are incompletely defined. In this study, a newly developed RF catheter system for transseptal left atrial entry was compared with conventional needle puncture. Of 15 piglets having transfemoral cardiac catheterization, 12 had transseptal procedures. Needle punctures (NP) and PRF were followed by acute (1 hr; 3 NP, 3 PRF) and chronic necropsy (1 month; 3 NP, 3 PRF). The remaining three piglets had intentional RF aortic perforation through the atrial roof with necropsy at 1 month. Gross and histopathological effects were examined. Acutely, the gross RF lesion was similar to needle puncture. Histologically, the RF lesions had minimal mural thrombus, an inner zone of thermal injury characterized by grayish cytoplasmic staining (elastic trichrome), and a bubbly transformation of the cytoplasm in innermost cardiomyocytes, partial persistence of cross‐striations, and an acute inflammatory reaction. The outer extent of the lesion (< 1 mm) was defined by a halo of contraction band necrosis similar to needle puncture. Acute NP injury showed comparable depth and extent of myocyte necrosis (principally contraction bands) with adjacent tissue hemorrhage and edema. At 1 month, a well‐developed densely collagenous scar was present in both aortic and transseptal PRF lesions. The extent of acute RF injury is similar to that seen in conventional NP, but the characteristics of tissue insult are different. Both show well‐developed healing at 1 month.

Native right ventricular outflow stent implantation in patients with unsalvageable extracardiac conduits

We describe two patients with unsalvageable ventricular‐to‐pulmonary artery conduits in whom endovascular stents were implanted in the native right ventricular (RV) outflow providing double outlet ventricle and RV hypertension relief. Both patients are free from surgical reintervention at 7 years and 5 months, respectively. Catheter Cardiovasc Interv 2004;61:256–258.

Percutaneous Closure of Postinfarction Ventricular Septal Defect

Background— Postinfarction ventricular septal defect carries a grim prognosis. Surgical repair offers reasonable outcomes in patients who survive a healing phase. Percutaneous device implantation represents a potentially attractive early alternative. Methods and Results— Postinfarction ventricular septal defect closure was attempted in 53 patients from 11 centers (1997–2012; aged 72±11 years; 42% female). Nineteen percent had previous surgical closure. Myocardial infarction was anterior (66%) or inferior (34%). Time from myocardial infarction to closure procedure was 13 (first and third quartiles, 5–54) days. Devices were successfully implanted in 89% of patients. Major immediate complications included procedural death (3.8%) and emergency cardiac surgery (7.5%). Immediate shunt reduction was graded as complete (23%), partial (62%), or none (15%). Median length of stay after the procedure was 5.0 (2.0–9.0) days. Fifty-eight percent survived to discharge and were followed up for 395 (63–1522) days, during which time 4 additional patients died (7.5%). Factors associated with death after postinfarction ventricular septal defect closure included the following: age (hazard ratio [HR]=1.04; P=0.039), female sex (HR=2.33; P=0.043), New York Heart Association class IV (HR=4.42; P=0.002), cardiogenic shock (HR=3.75; P=0.003), creatinine (HR=1.007; P=0.003), defect size (HR=1.09; P=0.026), inotropes (HR=4.18; P=0.005), and absence of revascularization therapy for presenting myocardial infarction (HR=3.28; P=0.009). Prior surgical closure (HR=0.12; P=0.040) and immediate shunt reduction (HR=0.49; P=0.037) were associated with survival. Conclusions— Percutaneous closure of postinfarction ventricular septal defect is a reasonably effective treatment for these extremely high-risk patients. Mortality remains high, but patients who survive to discharge do well in the longer term.Background— Postinfarction ventricular septal defect carries a grim prognosis. Surgical repair offers reasonable outcomes in patients who survive a healing phase. Percutaneous device implantation represents a potentially attractive early alternative. Methods and Results— Postinfarction ventricular septal defect closure was attempted in 53 patients from 11 centers (1997–2012; aged 72±11 years; 42% female). Nineteen percent had previous surgical closure. Myocardial infarction was anterior (66%) or inferior (34%). Time from myocardial infarction to closure procedure was 13 (first and third quartiles, 5–54) days. Devices were successfully implanted in 89% of patients. Major immediate complications included procedural death (3.8%) and emergency cardiac surgery (7.5%). Immediate shunt reduction was graded as complete (23%), partial (62%), or none (15%). Median length of stay after the procedure was 5.0 (2.0–9.0) days. Fifty-eight percent survived to discharge and were followed up for 395 (63–1522) days, during which time 4 additional patients died (7.5%). Factors associated with death after postinfarction ventricular septal defect closure included the following: age (hazard ratio [HR]=1.04; P =0.039), female sex (HR=2.33; P =0.043), New York Heart Association class IV (HR=4.42; P =0.002), cardiogenic shock (HR=3.75; P =0.003), creatinine (HR=1.007; P =0.003), defect size (HR=1.09; P =0.026), inotropes (HR=4.18; P =0.005), and absence of revascularization therapy for presenting myocardial infarction (HR=3.28; P =0.009). Prior surgical closure (HR=0.12; P =0.040) and immediate shunt reduction (HR=0.49; P =0.037) were associated with survival. Conclusions— Percutaneous closure of postinfarction ventricular septal defect is a reasonably effective treatment for these extremely high-risk patients. Mortality remains high, but patients who survive to discharge do well in the longer term. # CLINICAL PERSPECTIVE {#article-title-23}