Martin Kostolny

Presence of a Ventricular Septal Defect and the Mustard Operation Are Risk Factors for Late Mortality After the Atrial Switch Operation Thirty Years of Follow-Up in 417 Patients at a Single Center

Background— Survival and functional status of patients with transposition of the great arteries treated by atrial switch are reported to be reasonably good within the first 15 postoperative years. However, in some patients, the function of the systemic right ventricle deteriorates, leading to significant morbidity or even to late mortality. This study seeks to identify risk factors for late death. Methods and Results— Records of 329 patients after the Senning operation and 88 after the Mustard operation at a single center were retrospectively reviewed for demographic, anatomic, and echocardiographic predictors and outcomes. Mean follow-up interval was 19.1±6.5 years and was 95% complete. Survival 25 years after the Mustard procedure was 75.9±4.8% and after the Senning procedure was 90.9±2.3% (P=0.002). Mustard patients died more often of arrhythmia than Senning patients (P<0.001) and needed more baffle-related reoperations (P<0.0001). Ventricular septal defect closure at the time of the atrial switch operation (hazard rate=2.3; 95% confidence interval, 1.1 to 4.7; P=0.025) and the Mustard operation (hazard rate=2.0; 95% confidence interval, 1.01 to 3.8; P=0.045) emerged as independent risk factors for late mortality in multivariate analysis. At follow-up, 85.8% of the patients led a normal life with full-time work, and 11.8% were able to do part-time work. Only 2.4% experienced noticeable limitation of activities. Conclusions— Our patient data reveal satisfactory results at long term in this historic collective. Patients who had undergone ventricular septal defect closure at the time of the atrial switch operation and those who had undergone a Mustard operation are at higher risk for late death. Close follow-up, especially of these subgroups, is warranted.

Can we do without routine fenestration in extracardiac total cavopulmonary connections? Report on 84 consecutive patients

Fenestration is still widely used in right heart bypass operations. Our study was conducted to assess its need in the most recent modification, the completion of a total cavopulmonary connection with an extracardiac tube. The extracardiac approach was introduced at our institution in January, 1999. Since June of 2000, no patient had a fenestration. If more than 1 risk factor amongst ventricular function being more than moderately impaired, atrioventricular valvar regurgitation more than moderate, mean pulmonary arterial pressure more than 15 millimetres of mercury, mean atrial pressure higher than 12 millimetres of mercury, pulmonary arterial distortion, or other than sinus rhythm was present preoperatively, the patient was considered a high risk candidate. Postoperatively elevated pulmonary arterial pressure higher than 16 millimetres of mercury, prolonged effusions and requirement for drainage longer than 7 days, and death were considered endpoints in the statistical analysis. Our study group included 84 patients who underwent surgery up to August, 2004. A previous bidirectional cavopulmonary anastomosis had been accomplished in 73 patients at a mean age of 27.01 plus or minus 32.60 months, with a median of 11.5 months, without creating an additional source of flow of blood to the lungs. At the time of the total cavopulmonary connection, the mean age was 66.4 plus or minus 60.1 months, with a median of 37.1 months, and a range from 17.3 to 251.2 months, with 50 patients being younger than 48 months. We deemed 16 patients to be at high risk. These patients were older at the time of bidirectional cavopulmonary anstomosis (p smaller than 0.016), at the time of completion (p smaller than 0.019), and also differed in size at time of completion (p smaller than 0.020). They required a longer time on cardiopulmonary bypass (p smaller than 0.015), and reached higher early postoperative pulmonary arterial pressures after completion (p smaller than 0.025). There were no differences between groups of patients having up to 1 or more risk factors in regard to need for intubation (p smaller than 0.511), pulmonary arterial pressures after extubation (p smaller than 0.817), and duration of chest drainage (p smaller than 0.650). Three patients died, one in the group deemed at high risk. There was no death in the last 38 patients. We conclude that a total cavopulmonary connection with an extracardiac tube can be performed without fenestration, even if the patients are deemed to be at increased risk. Early staging of patients with functionally univentricular physiology might be one of the keys for these findings.

What was the impact of the introduction of extracardiac completion for a single center performing total cavopulmonary connections

BACKGROUNDnCreation of an extracardiac cavopulmonary connection has been proposed as a superior alternative to the lateral intracardiac tunnel for the completion of total cavopulmonary connection.nnnMETHODS AND RESULTSnWe made a retrospective review of our experience with 125 patients undergoing a total cavopulmonary connection between June 1994 and January 2003. Our experience with the extracardiac connection for completion began in 1999. Since 1994, we have constructed an intracardiac tunnel in 50 patients, and an extracardiac connection in 75. Of the total number, 83 had undergone an earlier partial cavopulmonary connection. Additional intracardiac procedures were performed in 43 patients at time of completion, in 25 of those undergoing extracardiac completion, and in 18 of the patients having an intracardiac procedure. The mean size of the tube used for completion was 19 mm. The mean cross-clamp time for placement of the intracardiac tunnel was 77 min, with a median of 80.5 min, and a mean cardiopulmonary bypass time of 139 min, with a median of 131 min. For construction of the extracardiac connection, a mean cross-clamp time in 24 of the 75 patients was 54 min, with a median of 54 min. Mean cardiopulmonary bypass time for all the patients with an extracardiac connection was 100 min, with a median of 88 min. Reoperations were needed in 10 patients, 6 having intracardiac and 4 extracardiac procedures. Of these, 5 were early and 5 late, including one take down. None of the patients died after these interventions. Taken overall, 8 patients died, with 5 early deaths. In the multivariable analysis, cardiopulmonary bypass time of more than 120 min, atrioventricular valvar replacement, and banding of the pulmonary trunk prior to the total cavopulmonary connection, all reached statistical significance for early death, whereas only heterotaxy syndrome remained as the sole risk factor for late death. There was no significant difference in survival between the modifications used.nnnDISCUSSIONnWhereas we could not identify any clinical superiority for the extracardiac approach in the short-term, the concept of extracardiac completion has helped to simplify the overall procedure. Longer follow-up will be required to elucidate any potential advantages.

Surgical removal of a rhabdomyoma in a neonate as rescue therapy

Primary cardiac and mediastinal tumors are rare at all ages. We report on a rescue therapy in a neonate due to a severely obstructed left ventricular outflow tract.

Temporary Pulmonary Vein Stenosis During Intraoperative Transesophageal Echocardiography in Total Cavopulmonary Connection

Two patients operated on by one of the authors (MK) developed hemodynamic instability after otherwise uneventful completion of total cavopulmonary anastomosis with an extracardiac tube. In both, a stenosis of the right pulmonary veins was demonstrated during routine intraoperative transesophageal echocardiography. The transesophageal probe was found to be the underlying problem. Apparently, the pulmonary veins became compressed between the probe and the extracardiac conduit.

Extended resection of a chest wall desmoid tumour with concomitant coronary artery bypass grafting.

We report on the resection of a large desmoid tumour of the anterior chest wall in a 65-year-old male patient. The patient had a coronary artery bypass operation 2 years prior to the first detection of a tumour. Because the left internal mammary artery bypass to the left anterior descending coronary artery (LAD) was embedded in the tumour mass, it had to be resected together with the tumour. A saphenous vein aorto-coronary bypass to the LAD with an off-pump technique was then performed, and the chest was reconstructed with polypropylene mesh and a latissimus dorsi musculocutaneous flap.

Surgical Management of an Extracardiac Total Cavopulmonary Connection in Heterotaxy Syndrome with Isolated Hepatic Drainage

The extracardiac modification for completion of a cavopulmonary connection has added a further option for direction of inferior vena cava and/or hepatic venous drainage to the pulmonary arteries. The authors describe a technique of isolating a hepatic vein and connecting it to the inferior caval vein in a side-by-side fashion prior to anastomosing it to the tubegraft in a patient with heterotaxy syndrome.ZusammenfassungFunktionell univentrikuläre Herzen können nach dem Fontan-Prinzip, in der Regel nach einem Stufenkonzept, einer kompletten Kreislauftrennung zugeführt werden. Neben der etablierten intraatrialen kavopulmonalen Verbindung ist eine extrakardiale Verbindung möglich. Vorteile eines extrakardialen Vorgehens sind u. a. Verzicht auf Ischämie (wenn keine intrakardialen Prozeduren nötig sind) und eine postulierte Reduktion des Auftretens von Arrhythmien im Verlauf (Vermeidung von atrialen Nahtreihen und atrialer Dehnung). Patienten mit Heterotaxiesyndrom und funktionell univentrikulärem Herz haben komplexe anatomische Verhältnisse und bedürfen besonderer Planung des operativen Vorgehens. Einem fehlenden oder unilateralen Lebervenenzustrom in das pulmonale Gefäßbett wird die Ausbildung intrapulmonaler arterio venöser Shunts zugesprochen. Berichtet wird über das chirurgische Vorgehen einer direkten Konnekion einer isoliert einmündenden Lebervene mit der unteren Hohlvene und der kavopulmonalen Komplettierung mit einem extrakardialen Rohr.

Successful primary correction of tetralogy of fallot with pulmonary atresia and aortopulmonary window in a 2,220-g neonate with a valved bovine jugular vein conduit.

Abstract.Prenatal diagnosis of tetralogy of Fallot with pulmonary atresia (TOF/PA) was confirmed in a newborn with a birth weight of 2,095 g. Additionally, an aortopulmonary window (APW) type I was diagnosed on echocardiography. The operation was performed at the age of 4 weeks due to congestive heart failure. The APW was closed with a pericardial patch, the ventricular septal defect (VSD) with a Dacron® patch, and the right ventricular outflow tract (RVOT) was reconstructed with a 12-mm bovine jugular vein valved conduit (Contegra®, Medtronic Inc., Minneapolis, MN, USA). At 15-month follow-up, the patient is in excellent clinical condition without medication. On echocardiography, the conduit showed a mean gradient of 11 mmHg with first-degree insufficiency.Zusammenfassung.Pränatal wurde die Diagnose einer Fallot-Tetralogie mit Pulmonalatresie gestellt. Bei Geburt, nach der 35. Schwangerschaftswoche, konnte echokardiographisch ein zusätzliches aortopulmonales Fenster festgestellt werden. Diese äußerst seltene Kombination wurde nach Wissen der Autoren in der Literatur erst sechsmal beschrieben. Der unrestriktive Shunt auf Höhe der großen Gefäße macht eine sofortige operative Korrektur zwingend notwendig. Trotz des geringen Körpergewichts gelangen der Anschluss an die Herz-Lungen-Maschine und die Durchtrennung der aortopulmonalen Verbindung. Nach Verschluss der aortalen Lücke mit einem autologen Perikardpatch wurde der Ventrikelseptumdefekt über eine kleine Ventrikulotomie mit einem Patch aus Dacron® verschlossen. Die Kontinuität zwischen dem Ausflusstrakt des rechten Herzens und den Pulmonalarterien wurde durch ein klappentragendes Conduit hergestellt. Nach 15 Monaten zeigt das Kind keine Gedeihstörungen und echokardiographisch einen mittleren Gradienten von 11 mmHg, bei eingradiger Insuffizienz, über das Conduit.Bei bekannter Knappheit besonders der kleinen Homografts wird seit wenigen Jahren von verschiedenen Gruppen als Implantat zur Rekonstruktion des rechtsventrikulären Ausflusstrakts die Jugularvene von Rindern (Contegra®, Medtronic Inc., Minneapolis, MN, USA) verwendet. Obwohl bisher überwiegend befriedigende Erfahrungen mit dem Implantat gemacht wurden, sollten nach ersten Beschreibungen von Klappenthrombosen oder überschießender Intimaproliferation längerfristige Erfahrungen an größeren Patientenkollektiven abgewartet werden.

Aortic Aneurysm 31 Years after Coarctation Repair with Direct Anastomosis

Background:Despite primary success, 9% of patients develop aortic (pseudo)aneurysms after surgical repair of aortic coarctation at or near the site of repair late after operation.Case Study:A chest X-ray in a 32-year-old asymptomatic man, 31 years after coarctation repair, depicted a ballooning of the distal aortic arch. A multislice CT confirmed an aneurysm. The aneurysm was resected using selective head perfusion.Conclusion:At follow-up of patients after coarctation repair, one should anticipate aneurysm formation, even decades after successful surgery and even if clinically silent. Therefore, the chest X-ray should be part of the follow-up examination. Deep hypothermic circulatory arrest can be avoided in selected patients.ZusammenfassungHintergrund:Nach Korrektur einer Aortenisthmusstenose werden pathologische Veränderungen der Aorta im Langzeitverlauf beobachtet.Fallbericht:In dem beschriebenen Fall wurde nach einer Röntgenuntersuchung eines asymptomatischen Patienten ein Aortenaneurysma vermutet. Die CT-Untersuchung bestätigte ein Aneurysma im Bereich der ehemaligen Anastomosenstelle. Unter Vermeidung eines totalen Kreislaufstillstands erfolgte nach selektiver Kopfperfusion die chirurgische Korrektur.Schlussfolgerung:Dieser Fall zeigt, dass die Langzeitbetreuung von Patienten mit Zustand nach operativer Korrektur einer Aortenisthmusstenose unerlässlich ist. Des Weiteren ermöglicht eine selektive Kopfperfusion ausgedehnte Eingriffe am Aortenbogen unter Vermeidung des totalen Kreislaufstillstands.