New developments in adult congenital heart disease.

Congenital heart disease (CHD) affects 0.8% of live births and over the past decades technical improvements and large-scale repair has led to increased survival into adulthood of over 95% of the new-born. A new group of patients, those who survived their congenital heart defect, has emerged but late complications including heart failure, pulmonary hypertension (PH), arrhythmias, aneurysms and endocarditis appeared numerous, with a huge impact on mortality and morbidity. However, innovations over the past years have changed the landscape of adult CHD dramatically. In the diagnostic process important improvements have been made in the use of MRI, biomarkers, e­health concepts and 3D visualisation of anatomy. Care is now concentrated in specialised centres, with a continuous emphasis on education and the introduction of weekly multidisciplinary consultations on diagnosis and intervention. Surgery and percutaneous intervention have been refined and new concepts applied, further reducing the burden of the congenital malformations. Research has matured from case series to global networks. Currently, adults with CHD are still facing high risks of early mortality and morbidity. By global collaboration and continuous education and development and innovation of our diagnostic and therapeutic arsenal, we will improve the perspectives of these young patients.

NT-proBNP and exercise capacity in adult patients with congenital heart disease and a prosthetic valve: a multicentre PROSTAVA study.

OBJECTIVES: N-terminal B­type natriuretic peptide (NT-proBNP) is an important biomarker for the detection of heart failure. Adults with congenital heart disease (ACHD) and a prosthetic heart valve are at risk for heart failure. This study aimed to determine the value of NT-proBNP in ACHD patients with a prosthetic valve and investigate its relationship with cardiac function and exercise capacity. METHODS: In this multi-centre cross-sectional observational study, data regarding medical history, echocardiography, exercise testing (VO2peak) and laboratory blood evaluation (including NT-proBNP) were collected in ACHD patients with a single prosthetic valve (either homografts, heterografts or mechanical valves). RESULTS: A total of 306 ACHD patients with pulmonary valve replacement (PVR, n = 139), aortic valve replacement (n = 141), mitral valve replacement (n = 21) or tricuspid valve replacement (n = 5) were investigated. The majority of patients (77 %) were in NYHA class I or II. Elevated NT-proBNP levels (cut-off ≥125 pg/ml) were found in 50 % of the patients, with the highest levels in patients with mitral valve replacements. In this study population, NT-proBNP levels were associated with gender (p = 0.029) and VO2max (p < 0.001). In PVR patients, NT-proBNP levels were associated with lower VO2peak, also after adjustment for age, gender and age at valve replacement in a multivariate model (p = 0.015). CONCLUSIONS: In patients with ACHD and a prosthetic valve, elevated NT-proBNP levels are frequently observed despite preserved NYHA class. In PVR patients, a higher NT-proBNP level was associated with a lower VO2peak. These results may be of importance in the ongoing discussion about the timing of valve replacement in patients with CHD.

Impact of introduction of 20-week ultrasound scan on prevalence and fetal and neonatal outcomes in cases of selected severe congenital heart defects in The Netherlands.

OBJECTIVES: To evaluate in a population-based cohort the effect of the introduction of the 20-week ultrasound scan in 2007 on the time of diagnosis, pregnancy outcome and total prevalence and liveborn prevalence of cases with selected congenital heart defects (CHDs) in The Netherlands. METHODS: We included children and fetuses diagnosed with selected severe CHD, born in the 11-year period from 2001 to 2011. Two groups of CHD were defined: those associated with an abnormal four-chamber view at ultrasound (Group 1), and those associated with a normal four-chamber view at ultrasound (Group 2). The time of diagnosis, pregnancy outcome and total liveborn prevalence were compared for both groups over two 5-year periods, before and after the introduction of the 20-week ultrasound scan. Trends in total and liveborn prevalence were examined over 2001 to 2011. RESULTS: Information was collected on 269 children and fetuses. After the introduction of the 20-week ultrasound scan, the prenatal detection rate of CHDs increased in both groups (Group 1, 34.6% in 2001-2005 vs 84.8% in 2007-2011 (P < 0.001); Group 2, 14.3% in 2001-2005 vs 29.6% in 2007-2011 (P = 0.037)). The rate of termination of pregnancy (TOP) increased significantly only for Group 1 (15.4% vs 51.5% (P < 0.001)). The total prevalence of CHD in Group 1 increased over time from 2.9 per 10 000 births in 2001 to 6.4 per 10 000 births in 2011 (P = 0.016). The liveborn prevalence did not show a trend over time. For Group 2, no trends in total or liveborn prevalence could be detected over time. CONCLUSIONS: Since the implementation of the routine 20-week ultrasound scan in The Netherlands, prenatal detection rate of selected severe CHDs increased significantly. Improved prenatal detection was accompanied by a more than three-fold increase in TOP, although only in those CHDs with an abnormal four-chamber view at prenatal ultrasound.

Postoperative renal dysfunction and preoperative left ventricular dysfunction predispose patients to increased long-term mortality after coronary artery bypass graft surgery.

BACKGROUND: Both preoperative left ventricular dysfunction and postoperative renal function deterioration are associated with increased long-term mortality after cardiac surgery. The influence of preoperative left ventricular dysfunction on postoperative renal dysfunction and long-term mortality is not defined. METHODS: We collected data from 641 consecutive patients undergoing coronary bypass surgery with cardiopulmonary bypass in 1991 at our institution. Prospective follow-up was through to July 2004. RESULTS: In-hospital mortality was 2.7% (17 of 641). During follow-up, 248 (40%) patients discharged alive died (5 and 10 yr survival 90% and 70%, respectively). On univariate analysis, preoperative left ventricular dysfunction (ejection fraction <50%) and an increase in serum creatinine > or =25% in the first postoperative week were associated with long-term mortality. The associated mortality risk was additive in predominantly non-overlapping patients groups: the hazard ratio (HR) for renal function deterioration only was 1.41 [95% confidence interval (CI) 0.95-2.32, P=0.083; n=64] and for left ventricular dysfunction only 1.71 (95% CI 1.26-2.95, P=0.0026; n=73). In patients in whom both were present, HR was 3.23 (95% CI 2.52-20.28, P<0.0001; n=20). Although postoperative renal dysfunction was associated with left ventricular dysfunction (P=0.008), both left ventricular dysfunction and postoperative renal function deterioration were independently associated with long-term mortality on multivariate analysis, as were age and the use of venous conduits. CONCLUSIONS: Both postoperative renal function deterioration and preoperative left ventricular dysfunction independently identify largely non-overlapping groups of patients with increased long-term mortality after coronary bypass surgery. In the group of patients with both factors present, the mortality risks appear additive.

Changes in glomerular filtration rate after cardiac surgery with cardiopulmonary bypass in patients with mild preoperative renal dysfunction.

BACKGROUND: Cardiac surgery with cardiopulmonary bypass (CPB) is commonly perceived as a risk factor for decline in renal function, especially in patients with preoperative renal dysfunction. There are few data on the effects of CPB on renal function in patients with mild preoperative renal dysfunction. The purpose of this study was to evaluate renal function in patients with pre-existing mild renal dysfunction undergoing cardiac surgery with CPB. METHODS: In a multicentre study cohort we measured prospectively the glomerular filtration rate (GFR) by radioactive markers both before operation and on the 7th postoperative day in cardiac surgical patients with preoperative serum creatinine >120 micromol l(-1) (n=56). In a subgroup of patients (n=14) in addition to the GFR, the effective renal plasma flow (ERPF) and the filtration fraction (FF) were measured. RESULTS: While preoperative GFR [77.9 (25.5) ml min(-1)] increased to 84.4 (23.7) ml min(-1) (P=0.005) 1 week after surgery, ERPF did not change [295.8 (75.2) ml min(-1) and 295.9 (75.9) ml min(-1), respectively; P=0.8]. In accordance, the FF increased from 0.27 (0.05) (before operation) to 0.30 (0.04) (Day 7, P=0.01). CONCLUSION: Our results oppose the view that cardiac surgery with CPB adversely affects renal function in patients with preoperative mild renal dysfunction and an uncomplicated clinical course.

Hybrid approach for complex coronary artery and valve disease: a clinical follow-up study.

BACKGROUND: For patients suffering from complex coronary artery disease (CAD) with or without concomitant valve disease, no evidence is available in the current guidelines to propose a predefined treatment regimen. We sought to assess the clinical impact of an unconventional or extended definition of the hybrid approach that combines percutaneous coronary intervention (PCI) and cardiac surgery in subjects suffering from severe solitary CAD or combined with valve disease. METHODS AND RESULTS: Between July 2002 and August 2004, 18 consecutive patients with complex CAD with or without significant valve disease who qualified for a hybrid approach were enrolled in a clinical follow-up study. Four patients eventually did not complete the proposed interventions. One patient refused treatment after inclusion, one patient died before treatment could be undertaken and two patients died after surgery but before PCI. In the other 14 cases combined treatment was technically successful. After a mean follow-up period of 15alpha5 months two patients had died, one due to sudden cardiac death and one of a noncardiac cause. No other major adverse clinical events were reported. A marked increase in quality of life was reported in those alive. CONCLUSION: Hybrid approach had a favourable long-term outcome in patients with complex cardiovascular disease undergoing successful treatment; however, this was observed at the expense of significant periprocedural mortality in these high-risk subjects. Therefore we believe that hybrid approaches may provide an alternative for selected cases. (Neth Heart J 2007;15:329-4.).

Long-term outcome after heart transplantation performed in the University Medical Centre Groningen.

BACKGROUND: Ten years ago, there was a difference of opinion about the suitability of ventilated patients with end-stage cardiac failure for heart transplantation (HTX). Although guidelines at that time qualified mechanical ventilation as a contraindication, we thought those patients could be candidates for HTX. In the same period a number of other patients received a donor heart in our centre. In this article we describe the clinical course and survival after these procedures. METHODS: We performed a retrospective study using our post HTX database. All patients undergoing transplants in our hospital were selected. Patients underwent echocardiography, scintigraphy (MUGA), ergo-spirometry (VO2 peak), blood tests and completed a quality of life questionnaire (SF-36). All tests were completed in the 1st quarter of 2006. RESULTS: Eight patients were identified; three were mechanically ventilated at the time of HTX. All eight patients were treated according to the standard protocol. Repeated surveillance cardiac biopsies were taken. One patient died 3.5 years after HTX due to an acute myocardial infarction. Seven patients, including the three patients on a ventilator at the time of the HTX, are alive, resulting in a survival rate of 88%. The current median survival time is 126 months (range 55 to 184 months). All patients are in good cardiac condition. The SF-36 domains of social functioning and mental health show high scores, the average score of general health and vitality is moderate. CONCLUSION: Survival of our eight transplanted patients after a median period of ten years was 88%, which is at least comparable with data from larger series. This finding suggests that HTX can be performed effectively and safely in a low volume centre. The finding that all three patients on a ventilator prior to HTX are alive is remarkable. It appears that mechanical ventilation is not always an absolute contraindication for HTX.

Additional value of biplane transesophageal echocardiography in assessing the genesis of mitral regurgitation and the feasibility of valve repair.

To determine the additional diagnostic value of biplane transesophageal echocardiography (TEE) in patients undergoing mitral valve surgery, we studied 48 patients with severe mitral regurgitation. Transesophageal echocardiographic video recordings were reorganized in separate transverse and longitudinal sections to allow independent evaluation. Mechanism of mitral regurgitation and anatomic abnormalities of the mitral valve were assessed by all 3 transesophageal echocardiographic modalities and were related to surgical findings. Biplane TEE detected increased leaflet mobility with a sensitivity of 91% and a specificity of 84%, and restricted leaflet mobility with a sensitivity of 100% and a specificity of 97%. Biplane TEE was accurate in the diagnosis of most of the anatomic abnormalities associated with these mechanisms. However, the sensitivity for detecting subvalvular abnormalities (including papillary muscle abnormalities) was poor, and measurement of the annular diameter had a poor correlation with annular dilatation. Although the yield of biplane TEE was better than either transverse or longitudinal TEE alone, the differences did not reach statistical significance, because of the size of the patient group. The surgical procedure (either valve repair or replacement) was correctly predicted with transverse TEE in 71%, with longitudinal TEE in 69%, and with biplane TEE in 79% of the patients. All 3 transesophageal echocardiographic modalities are very capable of assessing the anatomic abnormalities and mechanism of mitral regurgitation, as well as predicting the feasibility of valve repair.

Left atrioventricular valve after surgical repair in atrioventricular septal defect with separate valve orifices ("ostium primum atrial septal defect"): an echo-Doppler study.

Left atrioventricular (AV) valve dysfunction is the most frequent major postoperative hemodynamic complication in patients with AV septal defect. The anatomy and function of the left AV valve were investigated in 64 patients with separate valve orifices (ostium primum atrial septal defect) who had survived corrective surgery. M-mode and cross-sectional echocardiograms of the left AV valve were obtained. Doppler flow tracings were obtained at the left AV valve orifice to determine if regurgitation was present. The findings were related to the position of the commissures between the leaflets, the size of the 3 leaflets and the position of the papillary muscles. Left AV valve regurgitation was present in 29 of 51 patients (57%). These patients had a significantly different left AV valve leaflet configuration, characterized by a large mural leaflet and a small inferior bridging leaflet. The size of the superior bridging leaflet is not a determinant factor. Thus, the configuration of the left AV valve in AV septal defect is related to the postoperative functional result. Awareness of the echocardiographic anatomy may influence the surgical approach to this defect.

Anatomic and functional "obstruction" of the outflow tract in atrioventricular septal defects with separate valve orifices ("ostium primum atrial septal defect"): an echocardiographic study.

Left ventricular (LV) outflow tract (OT) obstruction can be treacherous in any form of atrioventricular (AV) septal defect. The properties of the LVOT were investigated echocardiographically in 64 patients with separate valve orifices ("ostium primum atrial septal defect") who had survived corrective surgery. M-mode and cross-sectional echocardiographic (echo) images were made of the LVOT. The degree of malalignment of the aorta with the ventricular septum, the left atrium-aortic ratio, the fractional LV shortening and the diameter of the LVOT were recorded. Fixed anatomical obstruction was found in 3 patients, consisting of muscular bands or abnormal attachment of tension apparatus. Malalignment of the aorta with the ventricular septum was found in 62% of the patients. The diameter of the LVOT was smaller than that of the aortic root in 71% of the cases. The mean diameter of the LVOT was 92 +/- 27% (range 35 to 143%) of the aortic root diameter. Because its walls are mainly muscular, the LVOT constricts during systole. The mean end-systolic diameter of the LVOT was 77 +/- 22% (range 23 to 129%) of the aortic root diameter. Sequential measurements showed that the LVOT constricted gradually, but the velocity of constriction in patients with the most severe narrowing showed a distinct maximum in the first fifth of systole. In conclusion, a series of elements contribute to a potentially perilous arrangement of the LVOT in patients with AV septal defect. This intrinsically narrow tunnel was constricted during systole by its muscular walls.(ABSTRACT TRUNCATED AT 250 WORDS)

Efficacy of serial electrical cardioversion therapy in patients with chronic atrial fibrillation after valve replacement and implications for surgery to cure atrial fibrillation.

Chronic atrial fibrillation (AF) occurs often in the setting of mitral and aortic valve disease. Eventually, these patients undergo valve replacement which improves cardiac function but does not prevent AF. This study investigates which patient may benefit from additional surgery for the cure of AF performed in combination with valve surgery. Seventy-four patients were retrospectively included from our prospective database of patients referred for serial cardioversion therapy between 1986 and 1993. All these patients had chronic AF after valve replacement. After the first electrical cardioversion, patients did not receive antiarrhythmic drugs. Relapses were managed by repeated cardioversions, and then antiarrhythmic drugs were instituted. After a median follow-up of 7 years (range 1.3 to 23), 39 patients had intractable AF. Multivariate analysis revealed that patients with a history of chronic AF before surgery (risk ratio 5.4, confidence intervals 2.5 to 11.3, p = 0.0001) had a poor arrhythmia outcome. In addition, Kaplan-Meier survival analysis demonstrated a lower success rate (p = 0.0017) in patients with mitral valve disease than in those with aortic valve disease. Congestive heart failure (41% vs 6%, p = 0.0007) and cardiovascular mortality (23% vs 9%, p = 0.09) were seen most often in patients with an unsuccessful cardioversion strategy. Thus, patients scheduled for mitral valve surgery with a history of chronic AF should be considered candidates for additional surgery for AF concomitantly performed during valve surgery.

Influence of age on survival, late hypertension, and recoarctation in elective aortic coarctation repair. Including long-term results after elective aortic coarctation repair with a follow-up from 25 to 44 years.

The optimal age for elective repair of aortic coarctation is controversial. The optimal age should be associated with a minimal risk of recoarctation, late hypertension, and other cardiovascular disorders. The purpose of this retrospective study is to determine the actuarial survival after aortic coarctation repair 25 years or more after operation and to calculate the optimal age for elective aortic coarctation repair. From 1948 to 1966, 120 consecutive patients underwent aortic coarctation repair. Eighty-seven were male (72.5%). The mean age at operation was 15.5 years (SD +/- 9.1 years). Resection and end-to-end anastomosis was performed in 103 patients (85.8%). Early mortality occurred in 6 patients as a result of surgical problems, whereas late mortality in 15 patients was predominantly caused by cardiac causes. The mean follow-up period was 32 years (range 25 to 44.2 years). Ninety-two patients 96.8%) were in New York Heart Association class I. The probability of survival 44 years after operation was 73%. Patients younger than 10 years at operation had the highest probability of survival at 97%. Multivariate analysis produced age at operation as the only incremental risk factor for the occurrence of recoarctation, of late hypertension, and of premature death. So that these sequelae can be avoided, elective aortic coarctation repair should be performed around 1.5 years of age. At that age, the probability of recoarctation will have decreased to less than 3%, and the probability of upper body normotension and long-term survival will be optimal.

Arterial revascularization with the right gastroepiploic artery and internal mammary arteries in 300 patients.

From September 1989 to September 1992, the right gastroepiploic artery in combination with one or both internal mammary arteries was used as a graft in 300 patients who underwent coronary artery bypass grafting. The gastroepiploic artery was the primary choice in preference to the saphenous vein. The study comprised 263 men and 37 women, ranging in age from 31 to 77 years (median age 59 years). Thirty-nine patients (13%) underwent previous bypass procedures with autologous vein grafts. In 17 patients (5.7%) the gastroepiploic artery was used as a single graft. In 150 patients (50%) the gastroepiploic artery in conjunction with one internal mammary artery was used (in 6 patients combined with a vein graft). In 133 patients (44.3%) the gastroepiploic artery was used with both internal mammary arteries. Revascularization in nine patients (3%) was combined with another cardiac procedure; three aortic valve replacements, two mitral valve repairs, and four resections of a left ventricular aneurysm. Ten patients died in the hospital (3.3%; 70% confidence limits 2.3% to 4.8%); two of these patients had an infarction in the area revascularized by the gastroepiploic artery. At late follow-up, 0.5 to 39 months (mean 14 months) after the operation, we found no mortality. One patient with an occluded gastroepiploic artery graft underwent reoperation with the use of the right internal mammary artery. One patient underwent percutaneous transluminal coronary angioplasty of the right coronary artery after occlusion of the gastroepiploic artery. Elective recatheterization was done in 88 patients 1 to 25 months after operation (mean 10 months). Graft patency in gastroepiploic artery grafts increased steadily from 77% in the first semester of the study to 95% in the fourth semester and then equaled the patency of the internal mammary artery grafts (97%), which was almost constant during the whole period. We conclude that patency of the gastroepiploic artery was initially related to a "learning curve" but eventually equaled that of the internal mammary artery grafts. Furthermore, the gastroepiploic artery may well be the graft of choice in conjunction with the internal mammary arteries.

Growth of the hypoplastic aortic arch after simple coarctation resection and end-to-end anastomosis.

Surgical treatment of a hypoplastic aortic arch associated with an aortic coarctation is controversial. The controversy concerns the claimed need to surgically enlarge the diameter of the hypoplastic arch, in addition to resection and end-to-end anastomosis. The purpose of this prospective study is to determine the fate of the hypoplastic aortic arch after resection of the aortic coarctation and end-to-end anastomosis. Between July 1, 1988, and January 1, 1990, 15 consecutive infants less than 3 months of age with an aortic coarctation were evaluated echocardiographically. A Z-value was calculated, being the number of standard deviations the aortic arch differs from the expected value, derived from a control group. Eight of these 15 infants had a hypoplastic aortic arch with a mean Z-value of -7.14 +/- 1.39. The other seven infants had a "normal" aortic arch with a mean Z-value of -1.85 +/- 1.08. All 15 infants underwent simple coarctation resection and end-to-end anastomosis. Six months after operation the mean Z-value increased significantly in those with a hypoplastic arch to -1.08 +/- 0.69 (p less than 0.0001) and in those with a "normal" aortic arch to 0.106 +/- 0.99 (p = 0.004). No infant died in our series (0%; CL 0% to 12%) and a recoarctation developed once (12.5%; CL 2% to 36%). Therefore we believe that simple resection and end-to-end anastomosis is the operation of choice for aortic coarctation associated with a hypoplastic aortic arch despite the presence of a ventricular septal defect and that enlargement of the hypoplastic aortic arch is not necessary.

Repair of aortic coarctation in infants.

Fifty-three consecutive infants younger than 2 years underwent coarctation repair. A recoarctation occurred in 11 infants (21%). To determine variables associated with recoarctation, we entered preoperative and operative data into a multivariate stepwise logistic regression analysis. Patient weight was an incremental risk factor for recoarctation instead of age, in contrast to previously published studies. Furthermore, the residual gradient after the operation was a strong incremental risk factor. This risk factor was even more significant when expressed as a ratio of the systolic arm pressure, which takes background hemodynamics into account. Because weight is a more significant risk factor than age, we conclude that deferring operation is indicated only when the infant gains weight. Furthermore, a residual gradient is more important in the hemodynamic setting of a lower systolic arm pressure.

Exclusive use of arterial grafts in coronary artery bypass operations for three-vessel disease: use of both thoracic arteries and the gastroepiploic artery in 256 consecutive patients.

METHODS: From September 1989 to September 1994 we operated on a consecutive group of 256 patients with three-vessel disease in whom we used the right gastroepiploic artery together with both internal thoracic arteries. Vein grafts were not used in these patients. This population consisted of 233 men and 23 women whose ages ranged from 31 to 77 years (mean age 57.8 years). RESULTS: Hospital morbidity and mortality were not directly related to the use of the gastroepiploic artery. Patency of the anastomoses in a subgroup of 56 patients (22%) a mean of 16 months after the operation was 98% for the left internal thoracic artery, 96% for the right internal thoracic artery, and 88% for the gastroepiploic artery. Five-year actuarial survival (including in-hospital deaths) was 95.9% and was related only to age. From discharge until the end of follow-up, two patients had a myocardial infarction, six patients underwent a reintervention procedure, and 18 patients had a return of angina pectoris. CONCLUSION: We conclude that the concomitant use of the gastroepiploic artery with the both internal thoracic arteries has low morbidity and mortality in patients with three-vessel disease operated on by experienced surgeons. At this moment, we have no reason to believe graft patency will deteriorate in the future. On the basis of these results, the knowledge that arteries are to be preferred over veins for coronary bypass grafting, and the absence of a leg incision, we believe this operative technique is superior to the use of venous grafts.

Decision making for the surgical management of aortic coarctation associated with ventricular septal defect.

Coarctation of the aorta and associated ventricular septal defect may be repaired simultaneously or by initial coarctation repair with or without banding of the pulmonary artery. The question is whether specific preoperative criteria can enable the surgeon to choose the optimal surgical management. Between 1980 and 1993, 80 infants younger than 3 months with coarctation and ventricular septal defect were treated surgically. In 64 infants (multistage group), simple coarctation repair was performed through a posterolateral approach, with concomitant banding of the pulmonary artery in 10 infants. Twenty ventricular septal defects were closed as a secondary procedure and four were closed as a tertiary procedure. Sixteen infants (single-stage group) underwent one-stage repair through an anterior midline approach. The total in-hospital mortality rate was 7.5%. Freedom from recoarctation after 5 years was 91.3% in the multistage group versus 60.0% in the single-stage group (p = 0.018). Freedom from secondary ventricular septal defect treatment in the multistage group after 5 years was 40.7%, versus 100% in the single-stage group (p = 0.016). Thirty-seven ventricular septal defects (47.8%) closed spontaneously. In particular, the preoperative left-to-right shunt and extension of the perimembranous VSD into the inlet or outlet were risk factors for the need for eventual surgical ventricular septal defect closure after initial coarctation repair. On the basis of these two risk factors, the probability of the need for eventual surgical treatment of ventricular septal defect after initial coarctation repair can be calculated. This policy offers a well-considered choice between single-stage and multistage repair, weighing the risk of secondary ventricular septal defect treatment versus the risk of recoarctation. Finally, the number of surgical procedures per infant will be as low as possible.

Thoracotomy for postchemotherapy resection of pulmonary residual tumor mass in patients with nonseminomatous testicular germ cell tumors: aggressive surgical resection is justified.

In patients with disseminated nonseminomatous testicular germ cell tumors (NSTGCT), a retroperitoneal residual tumor mass (RRTM) and/or a pulmonary residual tumor mass (PRTM) are often present after successful treatment with cisplatin-based polychemotherapy. Results and complications of postchemotherapy resection of PRTM were studied and survival was calculated. In the period 1979 to 1996, 31 patients with a median age of 28 years (range, 17 to 44 years) underwent 32 thoracotomies for the resection of a PRTM. A solitary lesion was encountered nine times (28.1%) and multiple lesions were encountered 23 times (71.9%). The median size was 15 mm (range, 2 to 60 mm). There were only three major postoperative complications (9.6%): prolonged ventilation, pneumothorax, and pneumonia. In 16 patients (51.6%), the resected PRTM showed mature teratoma, while in four patients (12.9%) it showed viable cancer. In 11 patients only necrosis and/or fibrosis were found (35.5%). Resection of an RRTM had been performed prior to thoracotomy in 20 patients. There was dissimilarity between the histologic features of the resected RRTM and PRTM in 10 of the 20 patients (50%). During a median follow-up of 80 months (range, 2.5 to 203 months), five patients died from metastatic disease (16.1%). The 5-year survival rate was 86.8% and the 10-year survival rate was 82.2%. Owing to the dissimilarity between the histologic features of the postchemotherapy resected RRTM and PRTM in 50% of the patients, all sites of pulmonary residual disease must be resected in patients with disseminated NSTGCT, irrespective of the histologic features of previously resected retroperitoneal residual disease. This approach offers minimal morbidity and a high 10-year survival rate.

Anomalies of the left atrioventricular valve and related ventricular septal morphology in atrioventricular septal defects.

A characteristic feature of atrioventricular septal defects is a deficiency of the inlet part of the ventricular septum that results in a "scooped out" appearance. The depth of the scoop in relation to the disposition of the atrioventricular valves has been debated. To clarify the relation between the morphology of the ventricular septum and the disposition of the atrioventricular valves, we quantified these anatomic features in 151 hearts at autopsy to determine whether those features identified particular groups within the overall lesion. We found that 137 hearts had left atrioventricular valves with three leaflets. The left valve in the other 14 hearts exhibited a dual orifice, a two-leaflet or one-leaflet arrangement, or was imperforate. These anomalies could be analyzed in terms of a sequence of diminishing formation of the commissures. Also, three-leaflet valves displayed a variability in which the angular size of the mural leaflet correlated negatively with that of the inferior leaflet. In some of the hearts with a common atrioventricular orifice, the bridging leaflets did not meet over the ventricular septum, thus creating a "gap." The mural leaflet's angular size corresponded to a deficiency of the combined inferior-mural leaflet complex. Hearts with an abnormal disposition of the left atrioventricular valve had the ventricular septum "scooped" to a greater extent than those with a common orifice, although most had separate right and left atrioventricular orifices.

Ventricular septal rupture caused by myocardial bridging.

A 70-year-old woman was referred to us with postinfarction angina. During cardiac catheterization the only coronary abnormality found was myocardial bridging in the mid and distal parts of the left anterior descending coronary artery, despite a large ventricular septal rupture. The pulmonary-to-systemic flow ratio was 2.5:1. Her operation was successful.