Adults With Congenital Heart Disease: Trends in Event-Free Survival Past Middle Age.

BACKGROUND: The survival of children with congenital heart disease has increased substantially over the past decades, with 97% currently reaching adulthood. The total effect of advanced treatment on future mortality and morbidity in adult survivors with congenital heart disease (CHD) is less well described. METHODS: We used data from the Swedish National Inpatient, Outpatient, and Cause of Death Register to identify patients with CHD who were born between 1950 and 1999 and were alive at 18 years of age. Ten controls identified from the Total Population Register were matched for year of birth and sex and with each patient with CHD. Follow-up was from 1968 and 18 years of age until death or at the end of the study (2017). Survival percentage with 95% CI for all-cause mortality were performed with Kaplan-Meier survival function. Cox proportional hazard regression models with hazard ratios (HRs) and 95% CI were used to estimate the risk of all-cause mortality. RESULTS: We included 37 278 patients with adult CHD (ACHD) and 412 799 controls. Mean follow-up was 19.2 years (±13.6). Altogether, 1937 patients with ACHD (5.2%) and 6690 controls (1.6%) died, a death rate of 2.73 per 1000 person-years and 0.84 per 1000 person years, respectively. Mortality was 3.2 times higher (95% CI, 3.0-3.4; P<0.001) among patients with ACHD compared with matched controls. Up to the maximum of 50 years of follow-up, >75% of patients with ACHD were still alive. Mortality was highest among patients with conotruncal defects (HR, 10.13 [95% CI, 8.78-11.69]), but also significantly higher for the more benign lesions, with the lowest risk in patients with atrial septal defects (HR, 1.36 [95% CI, 1.19-1.55]). At least 75% of patients with ACHD alive at 18 years of age lived past middle age and became sexagenerians. CONCLUSIONS: In this large, nationwide, register-based cohort study of patients with ACHD surviving to 18 years of age, the risk of mortality up to 68 years of age was >3 times higher compared with matched controls without ACHD. Despite this, at least 75% of patients with CHD alive at 18 years of age lived past middle age and became sexagenerians. A notable risk decline in the mortality for patients with ACHD was seen for those born after 1975.

Outcome and survival after open heart surgery for adults with congenital heart disease - a single center experience.

Introduction. Congenital heart disease (CHD) is the most common type of birth defect today. The adult congenital heart disease (ACHD) population is constantly growing and becoming older and more patients require cardiac surgery. The objective of this study was to review the surgical outcome of the open heart procedures performed on ACHD patients in the last 10 years at Sahlgrenska University Hospital (SUH) through a retrospective descriptive cohort study. Methods. A retrospective data collection was performed for 421 patients who underwent a total of 439 surgical procedures between 2009 and 2018 at the Cardiothoracic department in SUH. The primary outcomes were early (<30 days) and late survival. Secondary outcomes were postoperative complications and independent risk factors for postoperative complications. Results. 30-day mortality was 1.9%. Long-term survival after 3, 5 and 10 years were 96% ± 1, 94.3% ± 1.3 and 92.4% ± 1.8. 82 major complications occurred after 46 procedures (11.6%). The most common major complication was re-exploration due to hemorrhage. Risk factors for major complications were acute surgery and prolonged extracorporeal circulation time. 173 minor complications occurred after 90 procedures (22.5%). The most common minor complication was prolonged intensive care unit stay (>48 h). Conclusion. This study presents satisfactory early and midterm survival. The survival and frequency of major postoperative complications are well in line with what other studies have presented. Patients undergoing resternotomies had no increased risk for mortality or postoperative complications.

Survival in Children With Congenital Heart Disease: Have We Reached a Peak at 97%?

Background Despite advances in pediatric health care over recent decades, it is not clear whether survival in children with congenital heart disease (CHD) is still increasing. Methods and Results We identified all patients with CHD using nationwide Swedish health registries for 1980 to 2017. We examined the survival trends in children with CHD; we investigated the mortality risk in patients with CHD compared with matched controls without CHD from the general population using Cox proportional regression models and Kaplan-Meier survival analysis. Among 64 396 patients with CHD and 639 012 matched controls without CHD, 3845 (6.0%) and 2235 (0.3%) died, respectively. The mean study follow-up (SD) was 11.4 (6.3) years in patients with CHD. The mortality risk was 17.7 (95% CI, 16.8-18.6) times higher in children with CHD compared with controls. The highest mortality risk was found during the first 4 years of life in patients with CHD (hazard ratio [HR], 19.6; 95% CI, 18.5-20.7). When stratified by lesion group, patients with non-conotruncal defects had the highest risk (HR, 97.2; 95% CI, 80.4-117.4). Survival increased substantially according to birth decades, but with no improvement after the turn of the century where survivorship reached 97% in children with CHD born in 2010 to 2017. Conclusions Survival in children with CHD has increased substantially since the 1980s; however, no significant improvement has been observed this century. Currently, >97% of children with CHD can be expected to reach adulthood highlighting the need of life-time management.

The feasibility of left ventricular mechanical support as a bridge to cardiac recovery.

OBJECTIVE: To study the achievability of device weaning in patients receiving left ventricular assist devices (LVADs) as a bridge to transplantation. METHODS: Eighteen consecutive patients receiving a LVAD between September 1997 and June 2002 were included in the study. During a four-month follow-up, patients were repeatedly evaluated with right heart catheterization and echocardiography and, if functional improvement was observed, studied with the device turned off. Cardiac recovery was defined as off-pump LVEF>or=40% together with a significant improvement in invasive haemodynamic measurements (CI>or=2.5 and PCWP

Are biventricular assist devices underused as a bridge to heart transplantation in patients with a high risk of postimplant right ventricular failure?

OBJECTIVE: Right ventricular failure in patients treated using left ventricular assist devices is associated with poor outcomes. We assessed the strategy of preplanned biventricular assist device implantation in patients with a high risk for right ventricular failure. METHODS: Between 2010 and 2014, we assigned 20 patients to preplanned biventricular assist device and 21 patients to left ventricular assist device as a bridge to heart transplantation on the basis of the estimated risk of postimplant right ventricular failure. Preimplant characteristics and postimplant outcomes were compared between the 2 groups. RESULTS: Patients with a biventricular assist device were younger, more often female, and more frequently had nonischemic heart disease than left ventricular assist device recipients. At preoperative assessment, biventricular assist device recipients had poorer Interagency Registry for Mechanically Assisted Circulatory Support profiles, a lower cardiac index, and more compromised right ventricular function. Survival on device to heart transplantation/weaning/destination for biventricular assist device and left ventricular assist device recipients was 90% versus 86% (not significant), with shorter heart transplantation waiting times for biventricular assist device recipients (median days, 154 vs 302, P < .001). Overall survival at 1 year was 85% (95% confidence interval, 62-95) versus 86% (95% confidence interval, 64-95) (not significant). The majority of both biventricular assist device and left ventricular assist device recipients could be discharged to home during the heart transplantation waiting time (55% vs 71%, not significant), and complication rates on device were comparable between groups (major stroke 10% vs 10%, not significant). CONCLUSIONS: Planned in advance, the biventricular assist device seems to be a feasible option as bridge to heart transplantation for patients with a high risk of postimplant right ventricular failure. The outcomes for these patients were similar to those observed for contemporary left ventricular assist device recipients, despite those receiving biventricular assist devices being more severely ill.

Continuous improvement in outcome after heart transplantation - Long-term follow-up after three decades of experience.

AIMS: Heart transplantation (HTx) has become the standard treatment for patients with end-stage heart disease. We report on the long-term outcome after HTx at our centre and investigate trends in outcome over time. METHODS: During the period, between 1984 and 2014, a total of 610 HTx procedures were performed in 595 patients (median 48years; IQR 31-57years; range 24days-71years; mean 43years; 75% male) in our institution. Long-term outcome was investigated in the whole cohort, among children (n=76), bridged with mechanical circulatory support (MCS, n=131), re-transplanted (n=17), and concomitant kidney transplantation (n=12). RESULTS: Long-term survival was at 1, 5, 10, 15 and 20years: 86% (95CI 0.83-0.89); 77% (95CI 0.73-0.80); 63% (95CI 0.59-0.68); 48% (95CI 0.43-0.54) and 30% (95CI 0.25-0.36), respectively. The median survival for the whole cohort was 14.1years. Patients transplanted during the most recent time period (2010-2014) had a better survival compared to previous eras, with a 1- and 3-year survival of 94% (95CI 0.89-0.97) and 93% (95CI 0.88-0.96), respectively (p<0.001). However, when survival was analysed for long-term MCS (n=80) versus short term MCS (n=35), there was a significantly poorer survival for the short-term MCS group (p=0.001). Independent predictors of long-term mortality included recipient age (p=0.041); previous smoking (p=0.034); ischemic heart disease (p=0.002); and preoperative ventilator therapy (p=0.004). CONCLUSIONS: We have shown that continuous improvement in outcome after HTx still occurs. In the last time era, direct transplantation from short-term MCS was abandoned, which may have inflicted outcome during the last time era.

Surgical repair of post infarction ventricular septal defects: a national experience.

OBJECTIVES: Ventricular septal rupture is a rare but feared complication after acute myocardial infarction. Most reports about outcome after surgical treatment are single center experiences. We investigated the results after surgical repair in all patients in Sweden during a 7-year period. METHODS: All patients undergoing surgical repair 1992-1998 were identified with the aid of the Swedish Heart Surgery Registry. The patients (n=189, 63% men, mean age 69+/-8 years) were operated at 10 different centers. Pre-and peri-operative variables were collected from the Registry and individual patient charts. Mortality was calculated and a Cox proportional hazards regression model was used to identify independent predictors for early and late mortality. Mean follow-up was 2.4 years. RESULTS: Seventy-seven of the 189 patients died within 30 days (41%). Urgent repair (Risk Ratio 4.2 (2.0-8.9), P<0.001) and posterior rupture (RR 2.1 (1.3-3.4), P=0.002) were independent predictors of 30-day mortality. Total cumulative survival (Kaplan-Meyer) was 38% at 5 years. For patients that survived the first 30 days (n=112), 5 year cumulative survival was 67%. Independent predictors for mortality after 30 days were number of concomitant coronary anastomoses (RR 1.5 (1.2-2.0), P=0.001), residual postoperative shunt (RR 2.7 (1.4-5.4), P=0.004) and postoperative dialysis (RR 3.4 (1.5-7.5), P=0.003). CONCLUSIONS: Early mortality after surgical repair of post infarction septal rupture is still considerable. Early repair and posterior rupture are predictors of early mortality. Long-term survival in patients surviving the immediate postoperative period is limited by pre-existing coronary artery disease, postoperative renal failure and the presence of a residual postoperative shunt.

Extracorporeal membrane oxygenation as a bridge to lung transplantation: a long-term study.

OBJECTIVES: We investigated early outcomes in patients with end-stage pulmonary disease bridged with extracorporeal membrane oxygenation (ECMO) with the intention to perform lung transplantation (LTx). METHODS: ECMO was used as a bridge to LTx in 20 patients between 2005 and 2013. Most patients suffered from rapid progress of disease and most failed to stabilize on mechanical ventilation. Sixteen patients (10 males, median age 42 years, range 25-59) underwent LTx after ECMO support for a median of 9 (range 1-229) days. Most patients were not on the waiting list while receiving ECMO, but after being assessed were on the waiting list for a median of 6 (range 1-72) days before LTx or death occurred. Median follow-up at 535 (range 36-3074) days was 100% complete, 9 patients have been followed for >1 year and 4 patients have been bridged during 2013. RESULTS: Four patients died on ECMO waiting for a donor and as intention-to-treat, the success for bridging was 80% (16/20) and 1-year survival was 62% (10/16, not including 4 with <1-year follow-up). For those who underwent LTx, 3 patients died in-hospital after LTx on Days 0, 16 and 82, respectively, and currently, 11/16 (69%) are alive and 1-year survival for transplanted patients was 9/12 (75%). Median ICU stay before and after LTx was 9 (range 2-229) days and 20 (range 0-53) days, respectively. At follow-up, lung function was evaluated, and mean forced expiratory volume at 1 s and forced vital capacity were 56±22% of predicted and 74±24% of predicted, respectively. CONCLUSIONS: ECMO used as a bridge to LTx results in acceptable survival in selected patients with end-stage pulmonary disease.

The Berglin apical stitch: a simple technique to straighten things out in atrial fibrillation surgery.

In the Cox-Maze IV procedure, or in endocardial left atrial ablation, correct positioning of the surgical ablation probe within the left atrium might be difficult due to bulging or folds in the posterior left atrial wall. The Berglin apical stitch is a simple trick of the trade to create a smooth surface in the posterior left atrium that facilitates performing a safe transmural lesion and, consequently, may increase antiarrhythmic efficiency.

Early results in transplantation of initially rejected donor lungs after ex vivo lung perfusion: a case-control study.

OBJECTIVES: An increasing number of studies have shown that ex vivo lung perfusion (EVLP) is safe and that rejected donor lungs can be resuscitated and used for lung transplantation (LTx). Early clinical outcomes in patients transplanted with reconditioned lungs at our centre were reviewed and compared with those of contemporary non-EVLP controls. METHODS: During 18 months starting January 2011, 11 pairs of donor lungs initially deemed unsuitable for transplantation underwent EVLP. Haemodynamic (pulmonary flow, vascular resistance and artery pressure) and respiratory (peak airway pressure and compliance) parameters were analysed during evaluation. Lungs that improved (n = 11) to meet International Society of Heart and Lung Transplantation criteria were transplanted and compared with patients transplanted with non-EVLP lungs (n = 47) during the same time period. RESULTS: Donor lungs were initially rejected due to either inferior PaO2/FiO2 ratio (n = 9), bilateral infiltrate on chest X-ray (n = 1) or ongoing extra corporeal membrane oxygenation (n = 1). The donor lungs improved from a mean PaO2/FiO2 ratio of 27.9 kPa in the donor to a mean of 59.6 kPa at the end of the EVLP (median improvement 28.4 kPa, range 21.0-50.7 kPa). Two single lungs were deemed unsuitable and not used for LTx. Eleven recipients from the regular waiting list underwent either single (n = 3) LTx or double (n = 8) LTx with EVLP-treated lungs. The median time to extubation (12 (range, 3-912) vs 6 (range, 2-1296) h) and median intensive care unit (ICU) stay (152 (range, 40-625) vs 48 (range, 22-1632) h) were longer in the EVLP group (P = 0.05 and P = 0.01, respectively). There were no differences in length of hospital stay (median 28 (range 25-93) vs 28 (18-209), P = 0.21). Two patients in the EVLP group and 6 in the control group had primary graft dysfunction >Grade 1 at 72 h postoperatively. Three patients in the control group died before discharge. All recipients of EVLP lungs were discharged alive from hospital. CONCLUSIONS: The use of EVLP seems safe and indicates that lungs otherwise refused for LTx can be recovered and subsequently used for transplantation, although time to extubation and ICU stay were longer for the EVLP group.

Heparin does not improve graft function in uncontrolled non-heart-beating lung donation: an experimental study in pigs.

OBJECTIVES: Non-heart-beating donation (NHBD) has the potential to increase the number of patients treated with lung transplantation. Our study investigated, in a simulated clinical situation in the uncontrolled NHBD setting, whether or not heparin administration after death affects the donor lung function. METHODS: Twelve Swedish domestic pigs underwent ventricular fibrillation and were left untouched for 7 min followed by cardiopulmonary resuscitation with mechanical compressions for 20 min. The animals were declared dead after a 'hands-off' period of 10 min and randomized to heparin (300 IU/kg) or placebo given into a central venous catheter. In the animals receiving heparin, 2 more minutes of chest compression followed. Intrapleural cooling was initiated 1 h after death, and prevailed for 2 h. Ex vivo lung perfusion (EVLP) was performed with the Vivoline(®) system. Lung function was evaluated with blood gases at different oxygen levels, pulmonary vascular resistance (PVR), wet/dry weight ratio, macroscopic appearance and histology. RESULTS: During EVLP, there were no significant differences between groups in PaO(2) or PVR at any investigated FiO(2) level (1.0, 0.5 or 0.21). At FiO(2) 1.0 the PaO(2) in the heparin group was 64 ± 2 (range 57-73) kPa and in the non-heparin group 63 ± 4 (range 51-71) kPa. The values for PVR were 592 ± 90 (range 402-1007) and 647 ± 97 (range 426-1044), respectively. There was no significant difference between groups in wet/dry ratio or histology. CONCLUSIONS: The use of heparin is of no obvious benefit to the donor lungs in the uncontrolled NHBD situation. The exclusion of heparin will simplify lung donation from NHBDs.

Transplantation of initially rejected donor lungs after ex vivo lung perfusion.

OBJECTIVE: Ex vivo lung perfusion has the potential to increase the number of patients treated with lung transplantation. Our initial clinical experience with ex vivo lung perfusion is reviewed as well as early clinical outcome in patients transplanted with reconditioned lungs. METHODS: Six pairs of donor lungs deemed unsuitable for transplantation underwent ex vivo lung perfusion with Steen solution mixed with red blood cells to a hematocrit of 10% to 15%. After reconditioning, lung function was evaluated and acceptable lungs were transplanted. Technical experience with ex vivo lung perfusion as well as clinical outcome for patients transplanted with ex vivo lung perfusion-treated lungs were evaluated. RESULTS: Donor lungs initially rejected either as a result of an inferior partial pressure of arterial oxygen/ fraction of inspired oxygen (n = 5; mean, 20.5 kPa; range, 9.1-29.9 kPa) or infiltrate on chest radiograph (n = 1) improved their oxygenation capacity to a mean partial pressure of arterial oxygen/fraction of inspired oxygen of 57 ± 10 kPa during the ex vivo lung perfusion (mean improvement, 33.6 kPa; range, 21-51 kPa; P < .01). During evaluation, hemodynamic (flow, vascular resistance, pressure) and respiratory (peak airway pressure, compliance) parameters were stable. Two single lungs were not used for lung transplantation because of subpleural hematoma or edema. Six recipients from the regular waiting list underwent single (n = 2) or double (n = 4) lung transplantation. One patient had primary graft dysfunction grade 2 at 72 hours. Median time to extubation was 7 hours. All patients survived 30 days and were discharged in good condition from the hospital. CONCLUSIONS: The use of ex vivo lung perfusion seems safe and indicates that some lungs otherwise refused for lung transplantation can be recovered and transplanted with acceptable short-term results.

Does pretransplant left ventricular assist device therapy improve results after heart transplantation in patients with elevated pulmonary vascular resistance?

OBJECTIVE: Pulmonary hypertension (PH), defined as a pulmonary vascular resistance (PVR) >2.5 Wood units (WU) and (or) a transpulmonary gradient (TPG) >12 mmHg, is an established risk factor for mortality in heart transplantation. Elevated PVR in heart transplant candidates can be reduced using a left ventricular assist device (LVAD), and LVAD is proposed to be the treatment of choice for candidates with PH. We analyzed the effect on PVR of pretransplant LVAD therapy in patients with PH and compared posttransplant outcome with matched controls. Long-term survival was compared between heart transplant recipients with mild, moderate or severe PH and patients with no PH. METHODS: Heart transplant recipients 1988-2007 (n=405) were reviewed and divided into two groups with respect to pretransplant PVR: <2.5 WU (n=148) and >2.5 WU (n=158). From the group with PH, patients subjected to pretransplant LVAD therapy (n=11) were analyzed with respect to PVR at implant and at transplant and, with respect to outcome, compared to matched historical controls (n=22). Patients with PH without LVAD treatment (n=147) were stratified into three subgroups: mild, moderate and severe PH and survival according to Kaplan-Meier was analyzed and compared to patients with no PH. RESULTS: LVAD therapy reduced PVR from 4.3+/-1.6 to 2.0+/-0.6 WU, p<0.05. Three cases of perioperative heart failure required mechanical support whereas one control patient developed perioperative right heart failure requiring mechanical support. The incidence of other perioperative complications was comparable between groups. There was no difference in survival between LVAD patients and controls, 30-day survival was 82% and 91%, respectively and 4-year survival was 64% and 82%, respectively. CONCLUSIONS: Pretransplant LVAD therapy reduces an elevated PVR in heart transplant recipients, but there was no statistically significant difference in posttransplant survival in patients with PH with, or without LVAD therapy. The study revealed no differences in survival in patients regardless of the severity of the PH.

Health care consumption due to atrial fibrillation is markedly reduced by Maze III surgery.

BACKGROUND: Health care consumption and costs for the treatment of atrial fibrillation are high. Atrial fibrillation is effectively treated by the surgical Maze III procedure according to the Cox method. We describe the effects of this procedure on health care consumption and economy. METHODS: From October 1997 through March 2002, 72 patients underwent the Maze III procedure. Medical records of these patients were reviewed, and all data regarding hospitalization and outpatient clinic visits for atrial fibrillation and its related diseases were recorded. Accounting divisions from the contributing hospitals were consulted for the exact cost of each of these services, which were allocated into preoperative, perioperative, and postoperative periods. RESULTS: The perioperative mortality was zero. Long-term freedom from symptomatic atrial fibrillation was verified in 96% of the patients. The number of hospitalization days decreased by 84%, from 471 during the preoperative period to 79 in the postoperative (p < 0.001), and costs during the same periods decreased by 75%, from 7,075,000 Swedish Kronor to 1,757,000 Swedish Kronor (p < 0.001). CONCLUSIONS: The Maze III procedure significantly decreased the postoperative hospitalization costs in patients undergoing surgery primarily for atrial fibrillation. As well as providing an effective treatment for symptomatic arrhythmia, this procedure breaks the undesirable trend of increasing health care consumption resulting from treatment of atrial fibrillation.

Laser-Doppler flowmetry is reliable for early diagnosis of small-bowel acute rejection in the mouse.

The purpose of the study was to investigate intestinal mucosal perfusion in mouse small-bowel transplantation (SBT), using laser-Doppler flowmetry. Heterotopic SBT was performed in syngeneic and allogeneic combinations. Mucosal perfusion was measured both in the native and in the grafted intestine at time of surgery and at 1, 3, 6, and 8 days postoperatively. Histology specimens were obtained at the same time and graded for rejection. No rejection was seen in the syngeneic group at any of the time points studied. The allografts displayed significant decreased mucosal perfusion on postoperative days 3, 6, and 8. Rejection was histologically evident on postoperative days 6 and 8. Laser-Doppler perfusion in the rejecting intestinal allograft was decreased before onset of histological features of rejection. Mucosal blood flow measured by laser-Doppler could be used as an early indicator of acute rejection in SBT.