Rüdiger Lange

Mitral-valve replacement in children under 6 years of age

OBJECTIVE In patients with congenital mitral-valve disease, reconstructive surgery is the primary goal. However, in cases with severely dysplastic valves or failed repair, mitral-valve replacement (MVR) is the only option. We analyzed, retrospectively, data of 35 patients younger than 6 years of age, who underwent MVR at our institution. METHODS Between 1974 and 1997, 35 children underwent MVR. The ages ranged from 2.7 months to 5.5 years (mean=1. 9+/-1.7 years) and body weight varied between 3.2 and 16.7 kg (mean=8.2+/-4 kg). The main indication (57%) for valve replacement was severe mitral-valve insufficiency. Eighteen patients (51%) had undergone at least one previous reconstructive operation (mean=1. 46+/-1.86 years) before the MVR. In 29 cases (83%), mechanical prostheses were implanted. Six patients received a bioprosthesis. The size of the prostheses ranged between 14 and 27 mm. RESULTS The overall hospital mortality was 17.1% (6/35), and decreased from 33 (1974-1985) to 11.5% (1986-1997). Seven children died late. The actuarial survival after 20 years was 51.2+/-13.3%. Eight patients (23%) required 10 reoperations (8.2%/100 patient-years). Freedom from reoperation at 10 years was 50+/-22%. Valve-related complications were thrombo-embolism (n=2; 1.6%/100 patient-years), hemorrhage (n=1; 0.8%/100 patient-years), structural deterioration (n=3; 2.5%/100 patient-years) and non-structural dysfunction (n=3; 2. 5%/100 patient-years). Follow-up is 96% complete, with a total of 122 patient-years (mean=4.2+/-4.7 years). Eighty six percent of the patients are in New York Heart Association (NYHA) class I, 95% have sinus rhythm and 59% do not need medication. All survivors, except for those who received a bioprosthesis, were placed on a regimen of Phenprocoumon (Marcumar((R))), aiming to maintain the International Normalized Ratio (INR) between 2.5 and 3.5. In one third of these children, self-management of oral anticoagulation was performed either by the patients or their parents. CONCLUSIONS MVR in small children still carries a high risk. In our experience, the long-term results are satisfying. After failed reconstructive surgery, or as a primary procedure, we prefer mechanical prostheses. They are well tolerated and the incidence of anticoagulation-related complications is low.

Atrial fibrosis in heart surgery patients Decreased collagen III/I ratio in postoperative atrial fibrillation.

AbstractIn chronic atrial fibrillation, increased expression of angiotensinconverting enzyme (ACE) promotes upregulation of profibrotic proteins. Atrial fibrillation early after cardiac surgery (poAF) is common but unpredictable, and is regarded as a different entity. Therefore, the present study tested whether atrial expression of ACE, osteopontin (OPN), and collagen is elevated in patients with no history of AF but who develop poAF. Thus, 19 patients (66 ± 9 years) with postoperative sinus rhythm (poSR) were compared to 14 patients (68 ± 10) who experienced poAF. mRNA and protein expression were determined by RT–PCR and Western blotting. Picrosirius red was used to stain collagen. The medians for ACE, OPN, type I collagen (Col I), and type III collagen (Col III) mRNA and protein expression did not significantly differ between poSR and poAF (U-test). However, the Col III/I protein ratio was significantly lower in patients with poAF (2.62 vs. 1.09; poSR vs. poAF; p < 0.05). Our data suggest that the occurrence of poAF is not dependent on increased ACE and OPN expression, rendering the determination of preoperative OPN plasma levels inadequate to estimate the risk for the occurrence of poAF. Contrarily, a shift in atrial collagen expression levels in favor of Col I is linked to the occurrence of poAF.

Unloading of right ventricle by bidirectional superior cavopulmonary anastomosis in hypoplastic left heart syndrome patients promotes remodeling of systemic right ventricle but does not improve tricuspid regurgitation

OBJECTIVE To evaluate the influence of volume unloading by bidirectional superior cavopulmonary anastomosis on the systemic right ventricle in patients with hypoplastic left heart syndrome. METHODS A total of 90 consecutive patients with hypoplastic left heart syndrome, who had survived the early postoperative period after bidirectional superior cavopulmonary anastomosis, were studied. Seven patients were excluded because of tricuspid valve surgery before or in association with bidirectional superior cavopulmonary anastomosis. The echocardiograms of the remaining 83 patients were reevaluated for tricuspid valve regurgitation and the size of the tricuspid annulus before bidirectional superior cavopulmonary anastomosis and at the last available follow-up examination before total cavopulmonary connection. RESULTS Echocardiograms were performed a median of 5 days before bidirectional superior cavopulmonary anastomosis. Tricuspid valve regurgitation was graded as 0 in 11 patients, I in 37 patients, II in 24 patients, and III in 11 patients. Follow-up echocardiograms were performed a median of 17 months after bidirectional superior cavopulmonary anastomosis. Postoperatively, tricuspid valve regurgitation was graded as 0 in 14 patients, I in 37 patients, II in 21 patients, III in 6 patients, and IV in 5 patients. Postoperatively, the mean Z value of the tricuspid annulus stayed the same in patients with significant tricuspid valve regurgitation (grade III or IV) after bidirectional superior cavopulmonary anastomosis but had decreased in the remaining patients. No significant change was seen in the level of tricuspid valve regurgitation after bidirectional superior cavopulmonary anastomosis compared with the preoperative data. CONCLUSIONS The relative size of the tricuspid annulus in patients with hypoplastic left heart syndrome decreases after bidirectional superior cavopulmonary anastomosis, most likely owing to volume unloading and promotion of the remodeling of the systemic right ventricle. However, this remodeling of the right ventricle does not improve the grade of tricuspid regurgitation.

Evolution of right ventricular size over time after tetralogy of Fallot repair: a longitudinal cardiac magnetic resonance study

Aims It is commonly believed that pulmonary regurgitation (PR) after surgical repair of tetralogy of Fallot (TOF) leads to progressive right ventricular (RV) enlargement. However, progressive RV dilatation has never clearly been documented in this patient population. Therefore, we studied the size of the RV over time in patients after surgical TOF repair. Methods and results Fifty‐one consecutive patients after surgical TOF repair underwent at least two cardiovascular magnetic resonance (CMR) exams using a single CMR scanner. Patients with RV outflow tract obstruction, interventions other than the initial repair and CMR exams with use of sedation were excluded. Three subgroups with different repair techniques were studied: transannular patch repair [n = 22, age 17 ± 10 years], subvalvular patch repair [n = 15, age 22 ± 8 years], or non‐patch repair/infundibulectomy (n = 14, age 28 ± 11 years). Right ventricular end‐diastolic volume index (RVEDVI) and PR fraction did not change during the 37 ± 21 months follow‐up between first and last CMR in the whole group (RVEDVI: 118 ± 23 mL/m2 vs. 119 ± 23 mL/m2, P = 0.720; PR fraction: 33% (23–40%) vs. 32% (24–39%), P = 0.268). RVEDVI remained stable in all subgroups (transannular patch: 120 ± 21 mL/m2 vs. 122 ± 22 mL/m2, subvalvular patch: 112 ± 23 mL/m2 vs. 111 ± 23 mL/m2, non‐patch: 123 ± 28 mL/m2 vs. 123 ± 23 mL/m2, P = 0.827). RVEDVI at last CMR did not differ between groups (P = 0.301). Conclusions This study shows no progression of RV dilatation in patients after surgical repair of TOF with moderately dilated RVs and significant PR during a 3‐year follow‐up. RV dilatation in our patient group seems to be independent from surgical repair techniques.

Mortality Following Congenital Heart Surgery in Adults Can Be Predicted Accurately by Combining Expert-Based and Evidence-Based Pediatric Risk Scores.

Objectives: Currently, there are few specific risk stratification models available to predict mortality following congenital heart surgery in adults. We sought to evaluate whether the predictive power of the common pediatric scores is applicable for adults. In addition, we evaluated a new grown-ups with congenital heart disease (GUCH) score specifically designed for adults undergoing congenital heart surgery. Methods and Results: Data of all consecutive patients aged 18 years or more, who underwent surgery for congenital heart disease (CHD) between 2004 and 2013 at our institution, were collected. We evaluated the Aristotle Basic Complexity (ABC), the Aristotle Comprehensive Complexity (ACC), the Risk Adjustment in Congenital Heart Surgery (RACHS-1), and the Society of Thoracic Surgeons (STS)–European Association for Cardiothoracic Surgery (EACTS) scores. The proposed GUCH score consists of the STS-EACTS score, the procedure-dependent and -independent factors of the ACC score, and age. The discriminatory power of the scores was assessed using the area under the receiver–operating characteristics curve (c-index). A total of 830 operations were evaluated. Hospital mortality was 2.9%. C-indexes were 0.67, 0.80, 0.62, 0.78, and 0.84 for the ABC, ACC, RACHS-1, STS-EACTS, and GUCH mortality scores, respectively. Conclusion: The evidence-based EACTS-STS score outperforms the expert-based ABC score. The expert-based ACC score is superior to the evidence-based EACTS-STS score since comorbidities are considered. Our proposed GUCH score outperforms all other scores since it integrates the advantages of the evidence-based EACTS-STS score for procedures and the expert-based ACC score for comorbidities. Evidence-based scores for adults with CHD should include comorbidities and patient ages.

Treatment of right ventricle to pulmonary artery conduit stenosis in infants with hypoplastic left heart syndrome

OBJECTIVES To determine the incidence of right ventricle-to-pulmonary artery (RV-PA) conduit stenosis after the Norwood I operation in patients with hypoplastic left heart syndrome (HLHS), and to determine whether the treatment strategy of RV-PA conduit stenosis has an influence on interstage and overall survival. METHODS Ninety-six patients had a Norwood operation with RV-PA conduit between 2002 and 2011. Details of reoperations/interventions due to conduit obstruction prior to bidirectional superior cavopulmonary anastomosis (BSCPA) were collected. RESULTS Overall pre-BSCPA mortality was 17%, early mortality after Norwood, 6%. Early angiography was performed in 34 patients due to desaturation at a median of 8 days after the Norwood operation. Fifteen patients (16%) were diagnosed with RV-PA conduit stenosis that required treatment. The location of the conduit stenosis was significantly different in the patients with non-ringed (proximal) and the patients with ring-enforced conduit (distal), P = 0.004. In 6 patients, a surgical revision of the conduit was performed; 3 of them died prior to BSCPA. Another 6 patients had a stent implantation and 3 were treated with balloon dilatation followed by a BSCPA in the subsequent 2 weeks. All patients who were treated interventionally for RV-PA conduit obstruction had a successful BSCPA. Patients who received a surgical RV-PA conduit revision had a significantly higher interstage (P = 0.044) and overall mortality (P = 0.011) than those who received a stent or balloon dilatation of the stenosis followed by an early BSCPA. CONCLUSIONS RV-PA conduit obstruction after Norwood I procedure in patients with HLHS can be safely and effectively treated by stent implantation, balloon dilatation and early BSCPA. Surgical revision of the RV-PA conduit can be reserved for patients in whom an interventional approach fails, and an early BSCPA is not an option.

False aneurysm origination from the proximal anastomosis of a right ventricular to pulmonary artery shunt following staged repair of hypoplastic left heart syndrome

The Norwood I operation, including placement of a shunt from the right ventricle to the pulmonary arteries, has been adopted by many surgeons for neonates with hypoplastic left heart syndrome. A three-year-old male who had undergone the Norwood I operation, and the Glenn operation, presented with a cervical pulsating tumor prior to the operation for total cavopulmonary connection. At the Glenn operation, the right ventricle to the pulmonary artery shunt was closed with a clip proximally, and the distal part was resected. Following the Glenn operation, the child had had recurrent deep sternal infections caused by Serratia marcescens. Cardiac catheterization showed a false aneurysm from the proximal shunt anastomosis. The bleeding after resternotomy was managed by initiating cardiopulmonary bypass via the groin vessels. Cerebral air embolies were prevented by systemic application of potassium, to achieve cardioplegic arrest during chest opening. The shunt was removed and the defect was closed. After the shunt was confirmed to be free from infection, a total cavopulmonary connection was performed after three days postoperatively. The case illustrates the management of retrosternal aneurysms during resternotomy in children.

[Rare case of enterovirus myocarditis with consecutive acute heart failure and subsequent "rescue therapy" with biventricular assist device].

Anamnese und Befunde: Eine 37-jährige Patientin wurde im kardiogenen Schock bei Multiorganversagen eingewiesen. Echokardiographisch war die linksventrikuläre Funktion zunächst auf 35% reduziert. Klinisch bestand der V.a. eine Myokarditis. Anamnestisch bestand ein grippaler Infekt seit 1 Woche. Innerhalb von 24 h kam es zu einer weiteren dramatischen Reduktion der linksventrikulären Funktion auf 7%. Links- bzw. Rechtsherzkatheteruntersuchungen unter Entnahme von rechtsventrikulären Myokardbiopsien wurden durchgeführt. Die histo- und molekularpathologischen Untersuchungen ergaben die Diagnose einer Enterovirusmyokarditis.Therapie: Bei Multiorganversagen blieb als einzige Therapieoption die Implantation eines biventrikulären Unterstützungssystems („biventricular assist device“ [BVAD] – Berlin Heart).Verlauf: Postoperativ war die Patientin nur noch vorübergehend katecholaminpflichtig. Aufgrund der notwendigen Antikoagulation waren bei Nachblutungen drei Rethorakotomien erforderlich. Die Nieren- und Leberfunktion normalisierten sich im weiteren Verlauf. Das BVAD konnte schließlich nach 25 Tagen bei vollständiger Erholung der globalen Herzfunktion ohne Herz-Lungen-Maschine entfernt werden.Patient History and Findings: A 37-year-old woman was admitted in cardiogenic shock and multiorgan failure. On echocardiography, left ventricular function was reduced, at that time, to 35%. The patient had been suffering from a cold for 1 week prior to admission. Within 24 h, left ventricular function dramatically decreased to 7%. Examinations included leftand right-heart catheter evaluation with removal of right ventricular biopsies. Histology and molecular pathology revealed the diagnosis of an enteroviral myocarditis.Therapy: Imminently, a biventricular assist device (BVAD – Berlin Heart) was implanted.Postoperative Course: Postoperatively, only low-dose inotropic support was required. Under the requested anticoagulation, recurrent bleeding necessitated three rethoracotomies. Both renal and liver function normalized over time. On postoperative day 25, the BVAD was explanted without the need for cardiopulmonary bypass. Global ventricular function had normalized. The implantation of BVAD proved to be an efficient rescue therapy.

Predonation of autologous blood reduces perioperative allogenic transfusion requirement in grown-up patients with congenital heart disease

BACKGROUND Adults with congenital heart diseases have a substantial risk for bleeding upon re-operations. Due to the detrimental effects of allogeneic blood transfusion, reduction of transfusion requirement is a major concern. To investigate the efficacy of autologous blood predonation (ABP), we focussed on a homogeneous subgroup of patients, with right ventricular outflow tract reconstruction. METHODS Prospectively collected data included 76 patients older than 16 years with repeated right ventricular outflow tract reconstruction from May 1995 to November 2006. In 27 patients, ABP was performed without any complication. RESULTS Primary diagnoses included Tetralogy of Fallot in 50 patients and others in 26 patients. All patients had at least one previous operation, 62% had more than one. All patients received a homograft conduit between the right ventricle and the pulmonary artery. Preoperative haemoglobin was 123+/-15 g l(-1) in patients with ABP and 134+/-22 g l(-1) in the remainder (p=0.037), but was not significantly different after cardiopulmonary bypass until discharge from the intensive care unit. Significantly more patients without ABP required transfusion of allogeneic packed red cells (PRCs) (26 of 49 patients (53%) vs 4 of 27 patients (15%), p=0.001) and allogeneic fresh frozen plasma (FFP) (30 of 49 patients (61%) vs 6 of 27 patients (22%), p=0.002) than patients with ABP. Of 27 patients, 23 (85%) and 25 (93%) with ABP received their predonated PRC and FFP, respectively. Logistic regression analysis identified no ABP (p=0.005, odds ratio (OR) 5.4, 95% confidence interval (CI) 1.7-17.7) and time on extracorporeal circulation >83 min (p=0.009, OR 5.0, 95% CI 1.5-16.8) to be predictive for allogeneic blood transfusion. CONCLUSION ABP can be safely performed in grown-up patients with congenital heart disease without complications. Patients without predonation of autologous blood exhibit a fivefold increased risk for requiring allogeneic blood transfusion.

Minimalinvasive endovaskuläre Stent-Therapie bei Erkrankungen in der thorakalen Aorta

Die Inzidenz der Aortenerkrankungen nimmt aufgrund der Uberalterung der Bevolkerung stetig zu und hat sich innerhalb der letzten 20 Jahre von 2,9 auf 10,9 pro 100.000 Einwohner mehr als verdreifacht [1]. Bei Aortenerkrankungen muss zwischen Aneurysmen und Dissektionen unterschieden werden. Der Begriff Aneurysma (Abb. 50.1) bezeichnet die Ausweitung eines arteriellen Blutgefases. Sind dabei alle Wandschichten, also innere Schicht (Intima), mittlere Schicht (Media) und ausere Schicht (Adventitia) betroffen, so spricht man von einem echten Aneurysma (Aneurysma verum). Besteht die Aneurysmawand nur aus adventitiellem Gewebe, spricht man von einem falschen Aneurysma. Ursache fur ein Aneurysma ist eine Schwachung der elastischen Krafte der Media, die dann dem intravaskularen Druck nicht mehr standhalten kann.