Achievements in congenital heart defect surgery: a prospective, 40-year study of 7038 patients.

BACKGROUND: This article presents an update of the results achieved by modern surgery in congenital heart defects (CHDs) over the past 40 years regarding survival and the need for reoperations, especially focusing on the results from the past 2 decades. METHODS AND RESULTS: From 1971 to 2011, all 7038 patients <16 years of age undergoing surgical treatment for CHD at Rikshospitalet (Oslo, Norway) were enrolled prospectively. CHD diagnosis, date, and type of all operations were recorded, as was all-cause mortality until December 31, 2012. CHDs were classified as simple (3751/7038=53.2%), complex (2918/7038=41.5%), or miscellaneous (369/7037=5.2%). Parallel to a marked, sequential increase in operations for complex defects, median age at first operation decreased from 1.6 years in 1971 to 1979 to 0.19 years in 2000 to 2011. In total, 1033 died before January 1, 2013. Cumulative survival until 16 years of age in complex CHD operated on in 1971 to 1989 versus 1990 to 2011 was 62.4% versus 86.9% (P<0.0001). In the comparison of patients operated on in 2000 to 2004 versus 2005 to 2011, 1-year survival was 90.7% versus 96.5% (P=0.003), and 5-year cumulative survival was 88.8% versus 95.0% (P=0.0003). In simple versus complex defects, 434 (11.6%) versus 985 (33.8%) patients needed at least 1 reoperation before 16 years of age. In complex defects, 5-year cumulative freedom of reoperation among patients operated on in 1990 to 1999 versus 2000 to 2011 was 66% versus 73% (P=0.0001). CONCLUSIONS: Highly significant, sequential improvements in survival and reductions in reoperations after CHD surgery were seen. A future challenge is to find methods to reduce the need for reoperations and further reduce long-term mortality.

Preoperative versus postoperative survival in patients with univentricular heart: a nationwide, retrospective study of patients born in 1990-2015.

OBJECTIVES: Few data exist on mortality among patients with univentricular heart (UVH) before surgery. Our aim was to explore the results of intention to perform surgery by estimating preoperative vs postoperative survival in different UVH subgroups. DESIGN: Retrospective. SETTING: Tertiary centre for congenital cardiology and congenital heart surgery. PARTICIPANTS: All 595 Norwegian children with UVH born alive from 1990 to 2015, followed until 31 December 2020. RESULTS: One quarter (151/595; 25.4%) were not operated. Among these, only two survived, and 125/149 (83.9%) died within 1 month. Reasons for not operating were that surgery was not feasible in 31.1%, preoperative complications in 25.2%, general health issues in 23.2% and parental decision in 20.5%. In total, 327/595 (55.0%) died; 283/327 (86.5%) already died during the first 2 years of life. Preoperative survival varied widely among the UVH subgroups, ranging from 40/65 (61.5%) among patients with unbalanced atrioventricular septal defect to 39/42 (92.9%) among patients with double inlet left ventricle. Postoperative survival followed a similar pattern. Postoperative survival among patients with hypoplastic left heart syndrome (HLHS) improved significantly (5-year survival, 42.5% vs 75.3% among patients born in 1990-2002 vs 2003-2015; p<0.0001), but not among non-HLHS patients (65.7% vs 72.6%; p=0.22)-among whom several subgroups had a poor prognosis similar to HLHS. A total of 291/595 patients (48.9%) had Fontan surgery CONCLUSIONS: Surgery was refrained in one quarter of the patients, among whom almost all died shortly after birth. Long-term prognosis was largely determined during the first 2 years. There was a strong concordance between preoperative and postoperative survival. HLHS survival was improved, but non-HLHS survival did not change significantly. This study demonstrates the complications and outcomes encountering newborns with UVH at all major stages of preoperative and operative treatment.

Eight years of pulmonary valve replacement with a suggestion of a promising alternative.

UNLABELLED: In a retrospective study we assessed surgical results following right ventricular to pulmonary artery connection repair or replacement at a medium of 2.4 years (0-8) follow-up. Data were retrieved from hospital charts. RESULTS: Three hundred and sixty five operations were performed in 286 patients in eight years starting in 2000 using different surgical methods. Homografts and Monocusps had a more than 50% significantly lower risk for reoperation than Contegra or bicuspid valves (p < 0.01). Data for infants and older children and grown ups were analysed separately. In the infant group no significant difference between the different methods (homograft, Contegra and Monocusp) was detected. In older patients, the Perimount valves performed extremely well with no need for reoperation after 2.5 years of follow-up. Perimount valves and homografts performed better than other solutions (p = 0.01). CONCLUSION: Although the follow-up for the Perimount valves was short, they are promising and need to be followed long-term. The homograft and the Monocusp remain valuable choices.

Fluid accumulation in the staged Fontan procedure: the impact of colloid osmotic pressures.

OBJECTIVES: Despite Fontan surgery showing improved results, fluid accumulation and oedema formation with pleural effusion are major challenges. Transcapillary fluid balance is dependent on hydrostatic and colloid osmotic pressure (COP) gradients; however, the COP values are not known for Fontan patients. The aim of this study was to evaluate the COP of plasma (COPp) and interstitial fluid (COPi) in children undergoing bidirectional cavopulmonary connection and total cavopulmonary connection. METHODS: This study was designed as a prospective, observational study. Thirty-nine children (age 3 months-4.9 years) undergoing either bidirectional cavopulmonary connection or total cavopulmonary connection procedures were included. Blood samples and interstitial fluid were obtained prior to, during and after the preoperative cardiac catheterization and surgery with the use of cardiopulmonary bypass (CPB). Interstitial fluid was harvested using the wick method when the patient was under general anaesthesia. Plasma and interstitial fluid were measured by a colloid osmometer. Baseline values were compared with data from healthy controls. RESULTS: Baseline COPp was 20.6 ± 2.8 and 22.0 ± 3.2 mmHg and COPi was 11.3 ± 2.6 and 12.5 ± 3.5 mmHg in the bidirectional cavopulmonary connection group and the total cavopulmonary connection group, respectively. These values were significantly lower than in healthy controls. The COPp was slightly reduced throughout both procedures and normalized after surgery. The COPi increased slightly during the use of CPB and significantly decreased after surgery, resulting in an increased COP gradient and was correlated to pleural effusion. CONCLUSIONS: Fluid accumulation seen after Fontan surgery is associated with changes in COPs, determinants for fluid filtration and lymphatic flow. CLINICALTRIALS.GOV IDENTIFIER: NCT 02306057: https://clinicaltrials.gov/ct2/results?cond=&term=NCT+02306057.

Fluid accumulation after closure of atrial septal defects: the role of colloid osmotic pressure.

OBJECTIVES: Following paediatric cardiac surgery with cardiopulmonary bypass (CPB), there is a tendency for fluid accumulation. The colloid osmotic pressure of plasma (COPp) and interstitial fluid (COPi) are determinants of transcapillary fluid exchange but only COPp has been measured in sick children. The aim of this study was to assess the net colloid osmotic pressure gradient in children undergoing atrial septal defect closure. METHODS: Twenty-three patients had interventional and 18 had surgical atrial septal defect closures. Interstitial fluid was harvested using a wick method before and after surgery with CPB with concomitant blood samples. COP was measured using a colloid osmometer for small fluid samples. Baseline COP was compared with data from healthy children. RESULTS: COPp at baseline was 21.9 ± 2.8 and 21.4 ± 2.2 mmHg in the interventional and surgical groups, respectively, and was significantly lower than in healthy children (25.5 ± 3.1 mmHg) (P < 0.001). In the surgical group, the use of CPB significantly reduced COPp to 16.9 ± 2.9 mmHg (P < 0.001) and the colloid osmotic gradient [ΔCOP (COPp - COPi)] to 2.9 ± 3.8 mmHg (P < 0.001) compared with interventional procedure. One hour after the procedure, COPi was 15.6 ± 3.8 mmHg and 9.9 ± 2.1 mmHg (P < 0.001) and the ΔCOP was 5.4 ± 3.0 mmHg and 9.1 ± 3.1 mmHg (P < 0.003) in the interventional and surgical groups, respectively. CONCLUSIONS: Baseline COPp and COPi were lower in atrial septal defect patients compared with healthy children. The significantly lower COP gradient during CPB may explain the tendency for more fluid accumulation with pericardial effusion in the surgical group. The increased COP gradient after CPB may represent an oedema-preventive mechanism.

Cardiovascular surgery in Loeys-Dietz syndrome types 1-4.

OBJECTIVES: The first publication of Loeys-Dietz syndrome (LDS) described aortic rupture at young ages. Experience with new LDS types showed that the clinical course varies, and thresholds for prophylactic surgery are discussed. As this is an uncommon disease, experience needs to be shared. METHODS: Retrospective review of patients with LDS types 1-4 undergoing cardiovascular surgery during the years 1991-2016. RESULTS: Thirty-five patients (including 6 children with LDS2) underwent 57 operations. LDS 1, 2, 3 and 4 included 4, 17, 11 and 3 patients, respectively. Mean age at first surgery was 36 years, with a non-significant trend that LDS2 patients were younger. Of the 9 emergency surgeries, 7 were type A dissections, with 1 postoperative death. Twenty-two patients had prophylactic aortic root surgery (17 valve-sparing root replacements), with 1 postoperative death, 1 reoperation with valve replacement and 1 late death. Freedom from root reintervention and death was 92% at 13 years. Of the 11 patients with LDS3, 5 needed mitral valve surgery. Mitral valve disease was not found in the other LDS types. Ten patients needed >1 operation. Of the 57 operations, 33 were in the ascending aorta, 20 in the aorta distal to the arch including branches and 4 were isolated heart surgeries. Of the 20 vascular operations, 16 were in LDS2. Cumulative survival 20 years after first surgery (all patients) was 94.3%. CONCLUSIONS: Clinical course seems to be more aggressive in LDS2, with index operation at a younger age, and higher risk of needing several operations. Vascular disease distal to the arch is not uncommon. LDS3 seems to be associated with mitral valve disease. Prophylactic aortic root surgery is safe and durable.

[Transcatheter closure of atrial septal defects in the oval fossa]. / Kateterlukking av atrieseptumdefekter.

BACKGROUND: We report five years' experience with transcatheter closure of defects in the oval fossa at Rikshospitalet University Hospital in Oslo, Norway. MATERIAL AND METHODS: We reviewed the results in 69 patients between 1.3 and 74 years of age (median 5.3), weighing median 20 kg (7.8-109), with pulmonary blood flow 2.40.74 (1.3-4.8) times systemic flow, and median diameter of the defect 18 mm (9-34). The patients were followed clinically for one year. RESULTS: In 69/77 (90%) the defect could be closed. None had a significant residual shunt. One patient showed a very small residual leak after one year. Two children, not yet seen after one year, had a small shunt after one month. The complication rate has been low, and we have not been able to demonstrate any sequelae. The hospital stay is two nights without any convalescence period. The results compare favourably to surgery. INTERPRETATION: The method was efficient and safe in all age and weight groups. The very limited total volume of such procedures indicates they should be centralised and only carried out by a small number of physicians.

Single-center 50 years' experience with surgical management of tetralogy of Fallot.

OBJECTIVE: The aim of this study was to evaluate the long-term outcome of total repair for tetralogy of Fallot. We aimed to characterize late survival and the time-related risk of late reoperation. METHODS: Operative protocols, patient records, and the database of the department were evaluated from 1951 until 2008. The official death registry of Norway was used for follow-up. Of the patients identified, the follow-up was 99.6% complete. RESULTS: A total of 627 patients were studied. Of these, 570 could be identified for follow-up. There were a total of 41 early and 30 late deaths. The total early (including palliative procedures) mortality was 7.2% and total late mortality was 7.9%. However, during the last 10 years, no early mortality has been observed following repair. A total of 264 patients underwent some form of palliative procedure as their first treatment, and 541 patients had a reparative procedure performed, with an early mortality of 31 (5.7%). In patients subjected to a reparative procedure, there was no difference in freedom from death or reoperation following primary repair versus primary palliation. The use of transannular patch was associated with a highly significant risk of reoperation. CONCLUSIONS: Surgical treatment of the tetralogy of Fallot and related congenital cardiac malformations has good long-term prognosis. In this cohort of patients, more than one-third required additional procedures later on, and, in some cases, as many as four additional surgeries. Palliative procedures followed by repair do not influence survival or reoperation-free survival. There are no differences between transatrial versus transventricular repair on survival or re-repair. Any transannular incision increases the risk of re-repair, but does not influence long-time survival. There is an almost linear decrease in reoperation-free survival following any type of repair of tetralogy of Fallot, even for as long as 50 years since the first procedure.

Repair of complex left ventricular outflow tract obstruction in children.

BACKGROUND: Complex obstruction of the left ventricular outflow tract (LVOTO) in children may be treated with surgical procedures like the Ross-Konno or Koncz-Konno procedures, or modifications thereof. We present our results from the last 10 years. MATERIAL AND METHOD: During the period from December 1991 to April 2002, 17 patients/children with complex LVOTO were operated on. Patients treated with balloon valvuloplasty, open commisurotomy or simple valve replacement are excluded. The procedures performed were: Koncz-Konno (KK) or modified KK (n = 10), Ross-Konno (RK) (n = 2) and Ross (RO) (n = 5). Concomitant procedures included mitral valve replacement (n = 1), aortic arc plasty (n = 1), subvalvular myotomi (n = 1), resection of subvalvular membrane (n = 1) and aorto-coronary bypass (n = 1). Patients' ages ranged from 1 to 178 months, median 71 months. Body weights ranged from 3.8 to 50.0 kg, median 19.4 kg. Eight cases were redo procedures. RESULTS: Three early deaths occurred. One patient in the KK group, one in the modified KK group and one in the RK group. One patient died 8 years postoperatively of unknown reasons; no autopsy was performed. The rest of our patients who survived the initial major surgical treatment show normal physical performance and their quality of life seems to be good. CONCLUSION: Patients with complex LVOTO can be operated with biventricular repair in selected cases. Early and late mortality are acceptable.

A comparison of hospital costs with reimbursement received for patients undergoing the Norwood procedure for hypoplasia of the left heart.

OBJECTIVES: To determine whether the present system of reimbursement, based on diagnosis-related groups and regular financial budgeting, covers the costs incurred during hospitalisation of 7 children undergoing the three stages of the Norwood sequence for surgical treatment of hypoplastic left heart syndrome. METHODS: Between January and September 2003, 7 patients underwent initial surgical palliation with the Norwood procedure at the Rikshospitalet University Hospital. A prospective methodology was developed by our group to measure the costs associated with each individual patient. The patients were closely observed, and the relevant data was collected during their stay in hospital. The stay was divided into four different periods of requirements of resources, defined as heavy intensive care, light intensive care, intermediate care, and ordinary care. At each stage, we recorded the number of staff involved and the duration of surgery and other major procedures, as well as the cost of pharmaceuticals and other consumables. Based on these data, we calculated the cost for each patient. These costs were compared with the corresponding revenue received by the hospital for each of the patients. RESULTS: We found the total mean cost for the three stages of the Norwood sequence was 138,934 American dollars, while the corresponding revenue received by the hospital was 43,735 American dollars. During this period, one patient died during the first stage of the Norwood sequence. CONCLUSIONS: Our study shows that steps involved in the Norwood sequence are low-volume but high-cost procedures. The reimbursement received by our hospital for the procedures was less than one-third of the recorded costs.