Surgical outcome for congenital heart malformations in the adult age: a multicentric European study.

Congenital heart diseases are currently treated in the pediatric age. However, a conspicuous number of patients requires treatment in the adult age. This study has been undertaken by members of the European Congenital Heart Surgeons Association with the aim of evaluating the impact of cardiac surgery in this particular age group. We have collected data from 1,247 patients who underwent 1,287 operations during a 5-year period between January 1, 1997 and December 31, 2001. Patients were divided into three groups: (1) palliative procedures (4.4%), operation performed to improve patients' clinical status without restoring normal anatomy or physiology; (2) repair (79.3%), operation performed to achieve an anatomic or physiologic repair with separation of the pulmonary from the systemic circulation (included in this group are also Fontan-type repair and one and a half ventricle repair); (3) reoperation (16.3%), all the reoperations performed after repair (either anatomic or physiologic). Hospital mortality (within 30 days) was 2.4% (range, 0% to 15.3% in different centers). Kaplan-Meier estimates shows a 94% survival at 4 years, which is higher for repair (95%) as compared with reoperations (92%) or palliations (88%). Surgery for congenital heart disease in the adult age is a safe, beneficial, and low-risk treatment that modifies patients' natural history by improving their clinical status.

Compositional changes of a dicalcium phosphate dihydrate cement after implantation in sheep.

A hydraulic calcium phosphate cement having dicalcium phosphate dihydrate (DCPD) as end-product of the setting reaction was implanted in a cylindrical defect in the diaphysis of sheep for up to 6 months. The composition of the cement was investigated as a function of time. After setting, the cement composition consisted essentially of a mixture of DCPD and beta-tricalcium phosphate (beta-TCP). In the first few weeks of implantation, the edges of the cement samples became depleted in DCPD, suggesting a selective dissolution of DCPD, possibly due to low pH conditions. The cement resorption at this stage was high. After 8 weeks, the resorption rate slowed down. Simultaneously, a change of the color and density of the cement center was observed. These changes were due to the conversion of DCPD into a poorly crystalline apatite. Precipitation started after 6-8 weeks and progressed rapidly. At 9 weeks, the colored central zone reached its maximal size. The fraction of beta-TCP in the cement was constant at all time. Therefore, this study demonstrates that the resorption rate of DCPD cement is more pronounced as long as DCPD is not transformed in vivo.

Surgical treatment of complete A-V canal defects in children before 3 months of age.

OBJECTIVES: Surgical repair of complete A-V canal defects (CAVCD) is a well established procedure which is currently performed in infancy. The aim of this study is to evaluate surgical results of correction in early infancy in comparison to older age. METHODS: From January 1985 to March 2001, 119 consecutive patients (age range 27 days to 83 months, mean 6.7 months) underwent repair of CAVCD in our Institution. Forms with unbalanced ventricles in association with Fallot's tetralogy or heterotaxia were excluded from this series. Fifty-eight patients (49%) underwent correction before 3 months of age (Group A), and 61 patients (51%) after 3 months (Group B). Surgical repair was accomplished with a double patch technique in 100 patients (84%). Associated surgical lesions were treated simultaneously in 48 patients (40%). RESULTS: There were 11 operative deaths (<30 days) (two in Group A (3.4%) and nine in Group B (15%)) (P = 0.05). The remaining patients were discharged home in good haemodynamic condition. Reoperation for postoperative left A-V incompetence occurred in five patients in Group A and in eight patients in Group B. There were eight late deaths (three in Group A and five in Group B), of which four were non-cardiac related. At a mean follow-up time of 80 months (range 2-184 months) 100 patients are asymptomatic and well, and free from oral medication. Echocardiographic examination showed absent or mild residual left A-V valve incompetence in 91 patients (49 in Group A and 42 in Group B) and moderate left A-V valve incompetence in nine patients (four in Group A and five in Group B). Kaplan-Meier survival estimates at 10 years were 90% for Group A and 75% for Group B. Kaplan-Meier freedom from reoperation at 10 years was 89% for Group A and 84% for Group B. CONCLUSIONS: Our data demonstrate that repair of CAVCD under 3 months of age is the ideal approach to this malformation with a lower mortality rate at operation compared to older patients. Logistic analysis showed that an operative age >3 months is, compared to an age < or =3 months, an incremental risk factor for hospital mortality with an odds ratio of 4.8 (95% confidence limit 1-23.5) (P = 0.05). In the long term, freedom from reoperation for left A-V valve incompetence is higher when compared to children repaired at an older age.

Reoperations for acute prosthetic thrombosis and pannus: an assessment of rates, relationship and risk.

OBJECTIVE: Mechanical valvular prostheses have the advantage of longevity but carry a risk of thrombosis which is dependant on valve design, materials and host-related interface. While pannus is common to both biologic and mechanical valves, acute prosthetic thrombosis is mostly a complication of mechanical valves; therefore we investigated to find rates and risk of these obstructive complications. METHODS: Between 1/1/70 and 31/12/97, 2680 patients received at least one mechanical prosthesis in the aortic or mitral or tricuspid position and a total of 3014 operations were performed. Follow-up included 18523 years and was 98% complete. Incidence rates, Kaplan-Meier estimates, modeling of the hazard and multivariate analysis in the hazard domain were used in the analysis. RESULTS: Overall survival was 76%, 64%, 51%, 38.5% and 29% at 5, 10, 15, 20 and 25 years, respectively. It was significantly better in aortic than in mitral than in double prosthesis. 290 patients received a single reoperation, 37 a second, six a third and one a fourth reoperation. Two-hundred and fifty-one of these reoperations were exclusively due to malfunction of mechanical prosthesis, nine to malfunction of both mechanic and biologic prostheses. Most frequent reoperative indications was dehiscence (133), pannus (48) and thrombosis (29). The linearized rate of reoperations for pannus was 0.24%/patient per year, for valvular thrombosis 0.15%/patient per year. The shape of the thrombotic hazard was constant (at random) and the relative risk 12 times higher for tricuspid prosthesis, seven times higher for mitral prosthesis. Multivariate analysis controlling for prosthetic position, age, sex and prosthetic size, showed a 67% risk reduction with larger prosthesis (>27 mm), a 69% risk reduction with the Sorin tilting disk prosthesis and an 83% risk reduction with the bileaflet prosthesis. Pannus hazard shows a delayed exponential rise and was two times higher in tricuspid and three times higher in mitral position. Multivariate analysis showed a 50% risk reduction with larger prosthesis, an 11 times higher hazard of old (caged-disk, caged ball) prosthesis and a three times higher hazard of Lillehei-Kaster prosthesis. Reoperation for thrombosis has a 62% perioperative (30 days) survival compared to 92% survival of pannus reoperation. CONCLUSIONS: Mechanical valves have a low incidence of reoperation, mostly for prosthetic dehiscence. Pannus development is the next frequent complication increasing with time since implant, therefore in this series it was related to old valvular models and tilting disk prosthesis, with longer follow-up. Acute thrombosis occurs significantly earlier than pannus formation. Despite shorter follow-up we are therefore confident that bileaflet prostheses are less prone to this complication and pannus is a rare early etiologic factor. Thrombosis has very high operative risk as compared to pannus, justifying the present trend to thrombolysate selected cases.

Accuracy of electrocardiographic and echocardiographic indices in predicting life threatening ventricular arrhythmias in patients operated for tetralogy of Fallot.

OBJECTIVE: To validate the accuracy of the prognostic significance of non-invasive clinical diagnostic indices as predictors of sustained ventricular tachycardia (sVT) or fibrillation (VF) in patients undergoing repair for tetralogy of Fallot. METHODS: One way analysis of variance and pairwise comparison of the values with the Bonferroni correction, logistic multivariate analysis, and ordinal logistic analysis were used to study quantitative electrocardiographic and echocardiographic variables in 66 patients who had undergone surgery for tetralogy of Fallot by ventriculotomy at a mean (SD) age of 11.8 (9.5) years. The mean (SD) period of follow up was 16.1 (5.7) years after surgery. RESULTS: Four groups of patients were identified by ECG and 24 hour Holter monitoring: 19 (28.7%) without ventricular arrhythmias, 34 (51.5%) with minor ventricular arrhythmias, seven (10.6%) with non-sustained ventricular tachycardia (nsVT), and six (9.0%) with sVT or VF. One way analysis indicated significant differences in QT dispersion (QTd) and end diastolic volume of the right ventricle (EDVRV) among the groups. Univariate logistic analysis showed EDVRV, QTd, and QRS duration to be significantly associated with sVT or VF. Stepwise multivariate analysis and ordinal logistic analysis showed QTd to be preferable to QRS duration as an indicator, because it was unrelated to EDVRV, and was capable of separating different probability curves for nsVT as opposed to sVT or VF. CONCLUSIONS: Stratification of patients undergoing corrective surgery for tetralogy of Fallot and at risk of life threatening arrhythmias is possible by simple and inexpensive means, which provide sensitive and specific indices.

Late potentials and ventricular arrhythmias in arrhythmogenic right ventricular cardiomyopathy.

We studied 38 patients (mean age 32 +/- 14 years) with arrhythmogenic right ventricular cardiomyopathy (ARVC) to evaluate the clinical significance of histologic features on endomyocardial biopsy specimens as related to signal-averaged electrocardiography (SAECG), spontaneous ventricular arrhythmias, and hemodynamic features. Fifteen patients presented with ventricular tachycardia or fibrillation (sustained ventricular arrhythmias), 23 with other minor arrhythmias. SAECG variables and right ventricular ejection fraction (RVEF) were statistically correlated with the extent of myocardial fibrosis on biopsy in ARVC. An increased percentage of fibrous tissue (> or = 30%) was a significant univariate predictor of late potentials (p = 0.004) and reduced RVEF (p = 0.02). The 18 patients with late potentials had an increased percentage of fibrous tissue (p = 0.01), a reduced RVEF (p = 0.0004), and a higher risk for sustained ventricular arrhythmias (p = 0.05) than the 20 patients without late potentials. RVEF was the most powerful predictor of late potentials (p = 0.004) at multivariate analysis. Moreover, RVEF < or = 50% was associated with an increased risk for development of sustained ventricular arrhythmias (p = 0.02). A SAECG parameter, namely the root-mean-square voltage of the terminal 40 ms at 25 Hz, was an independent predictive factor for the occurrence of sustained ventricular arrhythmias (p = 0.02). Although fibrous tissue may contribute to delayed myocardial activation in ARVC, a reduced RVEF plays an essential role for spontaneous manifestation of sustained ventricular arrhythmias.

Prosthetic replacement of the tricuspid valve: biological or mechanical?

BACKGROUND: Incidence of tricuspid prosthesis replacement was 1.9% of all valvular operations performed between June 6, 1966 and April 18, 1996. Many series report similar figures, but institutional experience is limited and the consensus on treatment modalities is lacking. METHODS: One hundred tricuspid operations were performed on 83 patients (46 female). A primary operation was performed in 64 cases, 13 patients had one previous operation, 4 patients had two previous operations, and 2 patients had three previous operations. Seventeen patients required a tricuspid prosthetic valve rereplacement. There were 2 emergent and 17 urgent operations. The New York Heart Association class was IV in 13 patients (mean pulmonary artery pressure, 41 mm Hg), III in 66 patients (mean pressure, 38 mm Hg), and II in 21 patients. The most frequent operation was simultaneous replacement of the mitral and tricuspid valve (41 patients). Seventy biological and 30 mechanical prostheses were used. Total follow-up time was 613 years, mean 7.4 years (median 4.2 years), with a maximum of 27.8 years, and was 92% complete. RESULTS: Operative mortality was 24%. Survival was 0.54 (0.48 to 0.59, n = 39) at 5 years, 0.38 (0.32 to 0.44, n = 27) at 10 years, 0.31 (0.25 to 0.36, n = 19) at 15 years, 0.29 (0.23 to 0.34, n = 11) at 20 years, and 0.17 (0.098 to 0.26, n = 3) at 25 years. Early mortality was increased from higher New York Heart Association class (hazard ratio = 2.2), congenital disease (hazard ratio = 6.9), and valvuloplasty failure (hazard ratio = 4.3). The constant risk phase (4%/patient-year) after 2 years was enhanced by older operative age (hazard ratio = 1.4). Prosthetic type had no independent effect. Biological prostheses were at risk for 300 years and had a reoperation incidence of 4.7%/ patient-year (14 events); mechanical prosthesis were at risk for 137 years with a rate of 2.2%/patient-year (3 events) (p = 0.21). Three valve thromboses were observed in old-design mechanical prosthesis. Bioprosthetic degeneration showed a steeper rate after 7 years. CONCLUSIONS: This study does not show a clear superiority of biological versus mechanical prostheses. In the long run survival with mechanical prostheses could be superior, given the high rate of bioprosthetic degeneration after 7 years.

Multivariate analysis of survival after malfunctioning biological and mechanical prosthesis replacement.

BACKGROUND: Results of valvular reoperations depend on extrinsic and patients' intrinsic risk factors. New prosthetic substitutes continue to appear and the clinical effect is difficult to evaluate. Randomized studies are limited by patient selection and follow-up time. We followed the patient-centric outcome research applied to a large database of valvular operations. METHODS: Between January 1, 1970 and January 1, 1995 755 patients underwent one reoperation, 96 a second reoperation, and 12 a third reoperation. On January 1, 1996 a common closing date follow-up was obtained in 98.7% of reoperated patients. Multivariable analysis in the hazard domain was applied to obtain an upgradable model of survival that could be used for predictions and treatment comparison. RESULTS: Postoperative death hazard showed an early phase merging within 6 months with a constant low hazard phase. The survival proportion was 0.65 (70% CL, 0.63 to 0.67) at 5 years, 0.51 (70% CL, 0.49 to 0.53) at 10 years, 0.47 (range, 0.44 to 0.49) at 15 years, 0.42 (70% CL, 0.39 to 0.46) at 20 and 25 years. Significant incremental risk factors for early mortality were reoperative era 1970 to 1980 (hazard ratio = 2.8), reoperation number (hazard ratio = 1.9), heart penetration on surgery (hazard ratio = 7.6), emergent operation (hazard ratio = 5.8), urgent operation (hazard ratio = 2.1), prosthetic thrombosis (hazard ratio = 2.4), acute prosthetic endocarditis (hazard ratio = 3.0), acute endocarditis of the natural valve at antecedent operation (hazard ratio = 3.2), original floppy valve pathology (hazard ratio = 3.2), and mitroaortic replacement (hazard ratio = 5.7). Isolated mitral reoperation had a lower risk (hazard ratio = 0.5). Significant incremental risk factors for constant phase were: operative era (1970 to 1980) (hazard ratio = 2.0), congestive heart failure (hazard ratio = 2.6), reoperation on tricuspid valve after previous mitral insertion (hazard ratio = 4.9), reoperation for recurring dehiscence (hazard ratio = 4.6), double-valve procedure (hazard ratio = 1.6), coronary artery bypass graft (hazard ratio = 2.7), aortic root disease at original operation (hazard ratio = 2.1), older operative age (hazard ratio = 1.1). Use of bileaflet prosthesis was found to decrease significantly (p = 0.0002) the death risk (hazard ratio = 0.2). CONCLUSIONS: There is no late uprising hazard, and surviving patients remain exposed to a low risk of death (4% of patients per year). Considering simultaneously the confounding from operative age and operative era and the many concomitant risk factors, survival appears favorably influenced by use of bileaflet valves on reoperation.

Eisenmenger syndrome. Factors relating to deterioration and death.

AIMS: To assess the natural history, risk factors for death and deterioration of patients with Eisenmenger Syndrome. METHODS: The clinical course of 188 patients from three different cardiac centres specialized in adult congenital heart defects, followed for a median period of 31 years, was retrospectively analysed. According to the diagnosis, 64 males and 124 females, mean age at last follow-up 33.02+/-12.8 years, were divided into two groups: simple (128) and complex (60) congenital heart disease. Events analysed were: time variation of Ability Index, pulmonary and cerebral complications, non-cardiac surgery, urate metabolism and renal function, arrhythmias, pregnancy and contraception, medical therapy and transplantation, death from all causes and sudden death. RESULTS: Eighty-four percent of the overall population showed a satisfactory Ability Index (1 and 2) at the first attendance (median age 25 years). Patients with complex heart disease and Eisenmenger reaction had earlier clinical deterioration (18.6+/-11.3 vs 26.7+/-12.2) and shorter survival (25.8+/-7.9 vs 32.5+/-14.6 years). Thirty-eight (20.2%) patients had at least one episode of haemoptysis between 20 and 40 years of age but it did not modify overall survival; 25 (13.2%) had pulmonary thromboembolism at a mean age of 35.2+/-13.4 years. Fifteen (7.9%) had a stroke and 7 (3.7%) a cerebral abscess at a mean age of 31.4+/-15.7 and 24.1+/-4.9 years, respectively. Cerebral complications influenced the quality of life but did not modify survival. Patients who had venesection showed a 2.04 times greater hazard ratio for haemoptysis. Venesection did not reduce cerebral complications and in 20% caused anaemia and iron deficiency. Other non-cardiac surgery with general anaesthesia carried risks (23.5% of deaths). Significant maternal mortality (27%) in relation to pregnancy occurred with constant deterioration in physical status, high incidence of spontaneous abortions (35.8%) and cardiac abnormalities in offspring (20%). Sixty-one patients died during follow-up, mainly by sudden death (29.5%), heart failure (22.9%) and from haemoptysis (11.4%). Eight patients had heart and lung transplantation and five died 1 week to 4 years after transplant. Deterioration in Ability Index (worsening symptoms), age, complex defects, blood creatinine level, right ventricular dysfunction and non-cardiac surgery were variables which affected the prognosis adversely with uni- and multivariate analysis. CONCLUSIONS: patients with Eisenmenger syndrome can survive to the seventh decade with informed medical care and protection from special risks

Autotransplantation procedure for giant left atrium repair.

BACKGROUND: Giant left atrium has been associated with bronchopulmonary and left ventricular compression [Kawazoe 1983]. CASE REPORT: We present a patient with severe congestive heart failure (CHF), respiratory insufficiency and a giant left atrium (GLA) following two previous mitral valve procedures and tricuspid valve annuloplasty in the distant past. Mitral prosthetic function and ventricular systolic function were felt to be normal leading to a tentative diagnosis of diastolic restriction from left ventricular compression and pericardial constriction. A pericardial decortication procedure through left thoracotomy was initially done but proved ineffective. Subsequently, full evidence of hemodynamic failure due to the giant left atrium and its respiratory complication was recognized and the patient underwent cardiac autotransplantation procedure [Kosak 1987], with the aim to reduce the left atrial dimensions to normal. CONCLUSIONS: Calcification of posterior left atrial wall prevented a completely satisfactory reduction of atrial size and the severity of ventricular adhesions from the previous pericardial procedure resulted in very long cardiopulmonary bypass time with severe bleeding complications. This case provides ample evidence that GLA can cause respiratory failure and needs to be surgically corrected.

Repair of complete common atrioventricular canal defects in patients younger than four months of age.

BACKGROUND: The purpose of this study was to evaluate the impact of age at repair and atrioventricular (AV) valve anatomy on preoperative and postoperative AV valve incompetence (AVVR) was done to test the hypothesis that early repair (less than 4 months of life) can be safely accomplished and not only control heart failure but also improve surgical results on AV valve reconstruction. METHODS AND RESULTS: One hundred patients, median age 6.1 months, underwent repair of the complete common AV canal defect (CAVC) between 1981 and 1996. Surgery was performed in 37 patients (37%) less than 4 months of age (Group 1) and in 63 patients (63%) more than 4 months of age (Group 2). Surgical correction included double patch septal reconstruction in all. Trifoliate reconstruction of the left AV valve was selected in 93 patients (93%). Parametric time-related predicted survival was 92.9% at 14 years in Group 1 (70% confidence limits, 87.6% to 96.1%) and 75.9% at 15.4 years in Group 2 (70% confidence limits, 70.08% to 81.02%) (P=.038). Multivariate analysis in hazard function domain shows early repair as a negative risk factor for death (P=.038). Ordinal logistic regression equation indicates a higher probability of preoperative AVVR with older age at operation (P=.019). Regression analysis demonstrates good correlation between annular size and age at repair (r=.87, P < .01) and between annular size and AVVR (r=0.68, P < .01). Parametric time-related predicted freedom from reoperation was 82.7% at 15.4 years (70% confidence limits, 76.9% to 88.5%). Multivariate analysis in hazard function domain demonstrated Down's syndrome as a negative risk factor for reoperation (P=.05), whereas annular dilation increased the risk of this event (P=.027). CONCLUSIONS: Early correction of CAVCs is safe and beneficial not only in controlling chronic heart failure, but also in preventing annular dilation secondary to large QP/QS, as a potential mechanism of preoperative AVVR. Annular dilation is an incremental risk factor for reoperation. Early correction according to the double patch technique and trifoliate approach to the left AV valve reconstruction allows respect of valvar and subvalvar apparatus architecture, with a low incidence of postoperative AVVR, excellent survival rate, and low reoperative rate for residual AVVR.

Aortic dissection type A versus type B: a different post-surgical death hazard?

OBJECTIVE: Patients with type B aortic dissection differ from patients with type A dissection in age, hypertension prevalence, indications and timing of surgical treatment, yet reported long-term results have been rather similar (see Doroghazi et al. J Am Coll Cardiol 1984;3:1026-1034). METHODS: With the aim of comparing the post-surgical history, we have reviewed our results in 288 dissections, 213 type A and 75 type B, operated consecutively between 1 January 1970 and 31 November 1994. Follow-up was 100% complete. Empirical survival of both groups was interpolated with a fully parametric method and the shape and scale of the hazard function was investigated. RESULTS: Survival was not significantly different between type A and type B. Parametric survival was, respectively, 0.52% (70% C.L.: 0.48-0.55) vs. 0.56% (0.51-0.62) at 5 years, 0.44% (0.40-0.47) vs. 0.28% (0.23-0.25) at 10 years, 0.37% (0.33-0.41) vs. 0.25% (0.19-0.32) at 15 years, and 0.31% (0.26-0.35) vs. 0.24% (0.18-0.31) at 20 years. Following the high perioperative risk phase in type A dissection, the intermediate and late risk remains constant at a rate of 0.0033 events/month (3.9% patient-years (pt.-years)). By contrast, the postoperative course of type B dissection shows an intermediate risk phase between 4 and 10 years with an average linearized risk of 9.3% pt.-years and a peak of 20%. This determined lower survival rates (24 vs. 31% at 20 years, P = NS). CONCLUSIONS: We conclude that patients with type B dissection have a steeper postoperative death hazard as compared to type A dissection patients. Age confounding or late entry do not explain the difference. This could be possibly related to a greater propensity for expansion, higher risk of malperfusion complications or to limitations of our current surgical treatment.

Aorto-right atrial fistula: a rare complication of aortic dissection type A. A report of two cases.

BACKGROUND: Aortic dissection with rupture into the right atrium is an extremely rare condition and rapidly lethal. The authors report their experience in the treatment of two complicated aortic dissections observed at 85 and 93 months after previous cardiac operations. These were redo mitral valve replacement and saphenous vein coronary artery bypass grafts. METHODS: Both cases underwent hospital admission on an emergency basis because of severe chest pain and cardiac failure. One case had a history of hypertension. The aortography revealed aortic dissection and a fistulous communication into the right atrium. At surgery dissection repair and closure of the fistula was performed. Furthermore in one case vein grafts were reimplanted into the vascular graft. RESULTS: Both patients survived the operation without complications, were rapidly extubated and were asymptomatic and fully active at late follow-up. CONCLUSIONS: In the light of these results we conclude by affirming that aortic dissection with rupture into the right atrium is an extremely rare condition, rapidly lethal. Patient survival is strictly dependent on the time interval between fistulization of the dissection and the operation.

Should right-sided fibroelastomas be operated upon?

There is general agreement that left-sided elastomas must be removed to avoid systemic complications. Right-sided localization is less risky and surgical treatment is not agreed upon. We report a case of surgical resected fibroelastoma of the tricuspid valve and discuss the indications to surgery by reviewing the literature on the few reported cases. We feel that, if surgery is timely, the tumor can be resected, thus preserving the native valve. This provides an opportunity to prevent possible right-sided complications due to increasing tumor size and embolization.

Aortic dissection in puerperium: a case report.

An acute type A aortic dissection, complicated by massive aortic regurgitation, was diagnosed in the case of a 32-year-old woman, thirty-three days post partum. Emergency operation (Cabrol II) was performed with an uneventful postoperative course. We draw attention to this high risk event, which is particularly apt to involve women within a short time after an otherwise uneventful pregnancy.

Left atrioventricular valve incompetence after repair of common atrioventricular canal defects.

BACKGROUND: Reoperation rate for residual atrioventricular (AV) valve regurgitation after repair of common atrioventricular canal defect (AVCD) is currently between 5% and 10%. This retrospective analysis evaluates the impact of AV valve anatomy, age at repair, and surgical technique on postoperative AV valve incompetence. METHODS: Between January 1982 and July 1994, 205 patients with common AVCD underwent surgical repair at our institution. A complete form with common atrioventricular orifice (CCAVCD) was present in 81 patients. One hundred twenty-four had separate AV valves and orifices; 22 of these had a restrictive ventricular septal defect component. Preoperative moderate to severe AV valve regurgitation (AVVR) was present in 74 (36%), with a lower incidence in the CCAVCD group (20/81, 25%) versus the transitional (8/22, 36%) or the partial forms (46/102, 45%) (p = 0.02). The incidence was 12.5% (3/24) for CCAVCD patients less than 4 months of age (p = 0.02). Repair of the left AV valve was performed according to the trifoliate approach in most cases (142/205, 70%). Follow-up was 98% complete. RESULTS: By Kaplan-Meier analysis, survival at 12.3 years was 97.8% +/- 1% in partial AVCD, 95.4% +/- 4% in transitional AVCD, and 73.2% +/- 5% in CCAVCD (median follow-up, 60 months). Freedom from reoperation at 12.3 years was 93.5% +/- 2% for partial AVCD, 76.9 +/- 9% for the transitional form, and 68.3% +/- 5% for CCAVCD: Postoperative moderate to severe AVVR occurred in 42 patients (21%), with lower incidence for CCAVCD (10/81, 12.5%) versus transitional AVCD (8/22, 36%) and partial AVCD (24/102, 24%) (p = 0.02). Postoperative moderate AVVR was found in only 1 patient with CCAVCD less than 4 months of age (p < 0.01). Nine patients (5%) underwent reoperation for residual postoperative AVVR Valve repair was performed in all with no operative death. By Cox proportional risk multivariate analysis, preoperative AVVR and double orifice "mitral" valve were associated with increased risk of postoperative left AVVR (p < 0.01), whereas a bifoliate approach appeared to reduce the risk of this event in partial AVCD (p = 0.03). CONCLUSIONS: Postoperative AVVR is related to the type of anatomy of the AV valve, to the age at repair, and to the surgical technique employed. Residual AVVR can still be corrected with conservative techniques at low mortality rates. Early repair of common AVCD is associated with a lower incidence of preoperative and postoperative AVVR in CCAVCD and seems to prevent progression of annular dilation and preoperative AVVR in partial AVCD.

Aortic valve prosthesis and late survival of successfully repaired type A dissection.

Seventy-two operative survivors of acute type A dissection between 1973 and 1993 had a complete follow up with a 16 years actuarial survival of 57% and a reoperation free actuarial survival of 34%. Follow up was 100% complete and covered a total of 500.6 patients-years with a median of seven years. Distal tear location (proximal, medium or distal aortic arch) with retrograde dissection toward the aortic valve was the single most important predictor of late mortality with a relative risk of 4.4 (70%CL 2.4-8.2) (p = 0.016). Mortality rate of patients with an aortic valve prosthesis was 6.2%/patient-year vs. 1.7/patient-year without aortic valve prosthesis, with a relative risk of 3.4 (70%CL 2.0-5.8) (p = 0.02). This finding could be due to confounding of variables related both to death and need of aortic valve replacement. Therefore a multivariate proportional hazard analysis, with mandatory inclusion of possible confounders, was done. It showed that patients with aortic valvular prosthesis had eight times higher risk (70% CL 2.7-24.2); prosthetic aortic valves and conservative aortic root procedures had an unconfounded relative risk of 14 times higher (70%CL 3.4-58.7) (P = 0.06), whereas patients with a composite conduit had a six times higher risk (70%CL 2.0-19.9) (p = 0.11). These findings support the attitude favoring a selective approach to aortic root repair and oppose standardized use of aortic valve prosthesis or composite conduit in acute type A dissection.

Arrhythmogenic right ventricular cardiomyopathy in young versus adult patients: similarities and differences.

OBJECTIVES: This study was designed to evaluate and compare the patterns of arrhythmogenic right ventricular cardiomyopathy in young people and adults. BACKGROUND: Few data are available on this cardiomyopathy in young people because clinical and morphologic findings considered pathognomonic are normally based on observations in adults. However, a familial occurrence with a probable genetic transmission led to the study of children and to early detection of this disease, in which sudden death has been reported even in young people. METHODS: Seventeen young patients with arrhythmogenic right ventricular cardiomyopathy diagnosed at a mean age +/- SD of 14.9 +/- 4.9 years were studied. Clinical, electrocardiographic, echocardiographic, cineangiographic and biopsy findings were compared with those of 19 adult patients whose condition was diagnosed at a mean age of 38.1 +/- 13.4 years. RESULTS: Syncope occurred in 23.5% of the young patients but in none of the adults (odds ratio of familial sudden death 5.54, p = 0.1). Ventricular couplets (odds ratio 16.0, p = 0.004) and subtricuspid bulging on echocardiography (odds ratio 5.95, p = 0.036) were prevalent in the young group. Cineangiographic data in the two groups were similar, except that more hypokinetic areas were found in adults (odds ratio 4.44, p = 0.05). Morphometric analysis of biopsy sections showed a greater amount of fibrous tissue in the young patients (p = 0.04) and a prevalence of fatty tissue in the adults (odds ratio 12, p = 0.005). During an equivalent follow-up time (mean 7 years), two young patients died suddenly, and two had ventricular fibrillation in the absence of antiarrhythmic therapy. CONCLUSIONS: The pathognomonic criteria for the diagnosis of arrhythmogenic right ventricular cardiomyopathy in adults are also valid for young people. Sudden or aborted death occurred frequently in young untreated patients.

Propofol-fentanyl versus isoflurane-fentanyl anesthesia for coronary artery bypass grafting: effect on myocardial contractility and peripheral hemodynamics.

To avoid intraoperative awareness and postoperative respiratory depression from high-dose opioid anesthesia, propofol (P), or isoflurane (I) has been combined with moderate-dose opioid with varying results. However, the effects of both P and I on myocardial contractility and left ventricular afterload have not been completely quantified. The end-systolic pressure-diameter relationship (ESPDR) of the left ventricle (LV) is a reliable method to quantitatively assess LV contractility because it is relatively independent of changes in preload and incorporates afterload changes. The purpose of this study was to quantify the cardiodynamic effects of propofol-fentanyl (PF) anesthesia in comparison with isoflurane-fentanyl (IF) anesthesia in patients undergoing coronary artery bypass grafting (CABG). Thirty patients with normal or moderately impaired LV function (ejection fraction > or = 40% with LV end-diastolic pressure < or = 18 mmHg, no preoperative akinesia or dyskinesia) undergoing elective CABG were studied. After premedication with flunitrazepam, 2 mg orally, all patients were induced with thiopental, 1 mg/kg, fentanyl, 20 micrograms/kg, and vecuronium, 0.1 mg/kg, and were ventilated with oxygen/air (F(1)O2 0.6). Anesthesia was maintained throughout the procedure with a zero-order intravenous (IV) continuous infusion of P, 3 mg/kg/h (PF group), or with isoflurane inhalation of 0.6% (IF group), supplemented by intermittent boluses (5 micrograms/kg) of fentanyl (up to a total maintenance dose of 30 micrograms/kg). After intubation, a cross-section of the LV was visualized by two-dimensional transesophageal echocardiography and an m-mode echocardiogram was obtained at the maximum anterior-posterior diameter. The radial artery pressure tracing and the ECG were simultaneously recorded with the M mode.(ABSTRACT TRUNCATED AT 250 WORDS)