SARS-CoV-2 in a Neonate with Truncus Arteriosus: Management and Surgical Correction Timing.

Children seem to be less affected by SARS-CoV-2 infection. High risk categories should include patients with Congenital Heart Disease (CHD), both children and adults. We describe the case of a newborn with a postnatal diagnosis of Truncus Arteriosus (TA) type A1 without 22.q.11 deletion syndrome. Soon after birth, SARS-CoV-2 infection was transmitted by the father. Due to the onset of heart failure symptoms, diuretic therapy has been set up. For worsening of clinical conditions, inotropic support with milrinone was added. A progressive reduction of N-terminal-pro hormone BNP over the days has been observed. Fourteen days after the negativization of the nasopharyngeal swab, the patient underwent surgical repair with Cardiopulmonary Bypass (CPB). Postoperative course was not complicated and the patient was discharged in good clinical conditions. There is very little evidence suggesting the optimal timing for surgery in SARS-CoV-2 positive patients. With a lack of specific guidelines, current strategy suggests a symptom-based or a polymerase chain reaction (PCR) test-based approach. In our case it was challenging to determine COVID-19 impact on heart failure symptoms. Our case is the first describing the surgical correction of CHD in a 40 days year old patient, performed in CPB after 14 days from SARS-CoV-2 infection negativization.

Long-term assessment of clinical outcomes and disease progression in patients with corrected Tetralogy of Fallot.

OBJECTIVE: Understanding changes of right ventricular (RV) geometry and function in repaired Tetralogy of Fallot (rToF) patients can improve decision-making for pulmonary valve replacement. Therefore, we aimed to assess the magnitude and clinical correlations of RV changes in rToF patients. PATIENTS AND METHODS: Clinical and MRI data of rToF patients who underwent repeated cardiac magnetic resonance imaging (MRI) at two centers between December 2003 and September 2020 were analyzed together with anatomical factors, including RV outflow tract obstruction, pulmonary artery branch stenosis, and tricuspid regurgitation. Adverse cardiac events and/or NYHA class worsening were documented and correlated with MRI changes. QRS length was reported at each MRI. RESULTS: Two-hundred-and-nineteen rToF patients (53% males, aged 20.2 ± 10.1 years) were enrolled. An increase of ventricular dimensions, except LVEDVi, and worsening of right and left ejection fractions were found over an average period of 5 years of follow-up. These changes were statistically significant but within 10% of the initial value. No significant changes were reported on a year-to-year basis, except in a small group of patients (6%) in whom no predictive factors were identified. Despite similar RV dimensions at the first examination, younger patients had a higher RV ejection fraction and a different annual rate of change of ventricular dimensions compared to older ones. Patients with arrhythmias (20%) were more frequently older and had larger RV dimensions but showed no significant correlations with MRI changes/years. CONCLUSIONS:  Changes in RV dimensions and function occur rarely and very slowly in rToF patients. A small percentage of patients experience a significant worsening in a short time interval without any recognized risk factors. Arrhythmias appear to occur in a small percentage of cases in the late follow-up.

Reliability of Fetal Echocardiography in Predicting Postnatal Critical Hypoxia in Patients with Transposition of Great Arteries and Intact Ventricular Septum.

Critical hypoxemia soon after birth is the most critical preoperative determinant of neurological outcomes and survival in newborns with Dextro Transposition of the Great Arteries and Intact Ventricular Septum (D-TGAIVS). Our study aimed to define fetal echocardiographic aspects that can better predict neonates with D-TGAIVS at risk for restricted interatrial communication after birth. 31 fetuses with a prenatal diagnosis of D-TGAIVS were included in our study. We divided patients with D-TGAIVS according to the timing of balloon atrial septostomy: Urgent, Not-Urgent and no BAS. We identified five fetal echocardiographic aspects of the interatrial septum (redundant, aneurysmal, flat, fixed, hypermobile). No significant differences in these fetal echocardiographic features were found between the three different groups of D-TGAIVS according to the timing of balloon atrial septostmy. However, only two patients showed flat appearance of interatrial communication: both needed Urgent balloon atrial septostomy. The prevalence of hypermobile septum primum was significantly lower in the control group compared to patients with D-TGAIVS. Fetal echocardiographic aspects cannot predict patients with D-TGAIVS who will not need Urgent balloon atrial septostomy. Therefore, we recommended a delivery in a tertiary center, equipped for Urgent balloon atrial septostomy, for all patients with D-TGAIVS regardless of fetal echocardiographic features.

Determinants of functional capacity after mitral valve annuloplasty or replacement for ischemic mitral regurgitation.

OBJECTIVE: To identify the exercise echocardiographic determinants of long-term functional capacity, in patients with chronic ischemic mitral regurgitation, after restrictive mitral valve annuloplasty (RMA) or mitral valve replacement (MVR). METHODS: We retrospectively analyzed 121 patients with significant chronic ischemic mitral regurgitation, who underwent RMA (n = 62) or MVR (n = 59), between 2005 and 2011. Preoperatively, all patients underwent a resting echocardiographic examination, and a 6-minute walking test (6-MWT) to measure distance. Resting and exercise stress echocardiography, and the 6-MWT were repeated at 41 ± 16.5 months. RESULTS: After surgery, the 6-MWT distance significantly improved in the MVR group, and decreased in the RMA group (+37 ± 39 m vs -24 ± 49 m, respectively; P < .0001). Exercise indexed effective orifice area was significantly higher in the MVR, versus the RMA, group (MVR: change from 1.3 ± 0.2 cm(2)/m(2) to 1.5 ± 0.3 cm(2)/m(2); RMA: change from 1.1 ± 0.3 cm(2)/m(2) to 1.2 ± 0.3 cm(2)/m(2); P = .001). The mean mitral gradients significantly increased from rest to exercise, in both groups, but to a greater extent in the RMA group (change from 4.4 ± 1.4 to 11 ± 3.6 mm Hg; MVR: change from 4.3 ± 1.8 to 9 ± 3.5 mm Hg; P = .006). On multivariate analysis, MVR and exercise indexed effective orifice area were the main independent determinants of postoperative 6-MWT. In the RMA group, 25 patients experienced late mitral regurgitation recurrence, severe in 9 (14%) of them. The rate of postoperative cardiovascular events was significantly higher in the RMA group (21% vs MVR: 8%; P = .03). Follow-up survival was 83% in the RMA group and 88% in the MVR group (P = .54). CONCLUSIONS: For chronic ischemic mitral regurgitation, MVR versus RMA was associated with better postoperative exercise hemodynamic performance and long-term functional capacity.

Feasibility and safety of biventricular repair in neonates with hypoplastic left heart complex.

Hypoplastic left heart syndrome is a spectrum of structural cardiac malformations characterized by variable underdevelopment of the left heart-aorta complex. A minority of patients having a milder degree of left ventricular hypoplasia, described as hypoplastic left heart complex (HLHC), may be selected for biventricular repair. The objective of this study was to assess the outcome of the biventricular approach in HLHC. We evaluated retrospectively 30 neonates diagnosed with HLHC from the "12 de Octubre" University Hospital, following established criteria. We analyzed the echocardiographic data recorded just after birth and at last follow-up after surgery. All patients were operated on in the neonatal period using various surgical techniques. There were no early deaths and only 1 late death after a mean follow-up of 62.9 ± 43.8 months. All patients presented a significant growth of the left ventricular structures, with a Z-score increase of 1.17 ± 1.05 for mitral annulus, 1.72 ± 1.23 for aortic annulus, and 1.33 ± 1.46 for left ventricular end-diastolic diameter. Postoperatively, 18 patients showed a left valvular stenosis, and 17 patients underwent a reoperation and/or an interventional procedure. Freedom from surgery or interventional catheterizations at 1, 3 and 5 years was 53, 49 and 43%, respectively. The 29 current survivors are all in a good functional status. In our experience, we achieved good results from biventricular repair in patients with HLHC, with a significant growth of left heart structures and an excellent clinical status at a medium-term follow-up. Nevertheless, there was a high rate of reoperations and/or interventional catheterizations.

Identification of de novo mutations and rare variants in hypoplastic left heart syndrome.

Hypoplastic left heart syndrome (HLHS) is one of the most severe congenital heart malformations, characterized by underdevelopment of the structures in the left heart-aorta complex. The majority of cases are sporadic. Although multiple genetic loci have been tentatively implicated in HLHS, no gene or pathway seems to be specifically associated with the disease. To elucidate the genetic basis of HLHS, we analyzed 53 well-characterized patients with isolated HLHS using an integrated genomic approach that combined DNA sequencing of five candidate genes (NKX2-5, NOTCH1, HAND1, FOXC2 and FOXL1) and genome-wide screening by high-resolution array comparative genomic hybridization. In 30 patients, we identified two novel de novo mutations in NOTCH1, 23 rare patients inherited gene variants in NOTCH1, FOXC2 and FOXL1, and 33 rare patients mostly inherited copy-number variants. Some of the identified variations coexisted in the same patient. The biological significance of such rare variations is unknown, but our findings strengthen the role of NOTCH pathway in cardiac valve development, indicating that HLHS is, at least in part, a 'valve' disease. This is the first report of de novo mutations associated with isolated HLHS. Moreover, the coexistence of multiple rare variants suggests in some cases a cumulative effect, as shown for other complex disease.

Two-stage study (1990-2002) of North African immigrants in Italy.

The objective of the present study was to investigate the psycho-social health and weight status of two samples of North African immigrants measured in 1990 (166 males) and in 2000-2002 (173 males and females), respectively. In addition to the cross-sectional study, we conducted a repeated study on a sub-sample of 21 males measured both in 1990 and in 2000-2002. The study was carried out in Italian health and care dedicated centres spread all over the Bologna administrative areas, that belong to the AUSL (Azienda Unità Sanitaria Locale, Administrative Local Health Unit). To evaluate the health and weight status, we calculated the body mass index (BMI) and measured systolic and diastolic blood pressure. Questionnaires were used to assess the psycho-social status. The mean BMI increased significantly (p<0.05) after immigration, as the prevalence of overweight (BMI>25) was higher after a decade. There was a significant increase (p<0.001) in stress-related factors and in the desire to return home. We also recorded an increase in weight disorders, as the length of time since immigration was an important risk factor for overweight. Therefore, immigrants may have a high risk of obesity-related co-morbidities. However, the factors related to malnutrition and stress had only weak effects on blood pressure.

[Plastic bronchitis. A case report and review of the literature]. / Bronquitis plástica. A propósito de un caso y revisión de los casos asociados a cirugía de Fontan.

Plastic bronchitis is a rare complication in which bronchial casts of lymphatic origin develop in the tracheobronchial tree and cause airway obstruction. The main feature is expectoration of bronchial casts. This condition usually occurs in the postoperative period after the Fontan operation for congenital heart disease. Treatment modalities include mucolytics and chest physiotherapy, and in the most severe cases bronchoscopy to remove the casts and aerosolized urokinase or r-TPA. We describe a 12-year-old boy with pulmonary atresia with intact ventricular septum and severely hypoplastic right ventricle who underwent a modified Fontan operation at the age of 7 years. At the age of 12 years the patient started to present frequent episodes of coughing, dyspnea, and desaturation followed by difficult emission of white bronchial casts. An extracardiac Fontan conversion was performed to improve hemodynamics and symptoms. During the postoperative period frequent episodes of airway obstruction required bronchoscopy and finally improved with administration of aerosolized urokinase.

Bronquitis plástica. A propósito de un caso y revisión de los casos asociados a cirugía de Fontan / Plastic bronchitis. A case report and review of the literature

La bronquitis plástica es una rara manifestación consistente en la formación y expectoración de moldes bronquiales de origen linfático que provocan obstrucción de la vía respiratoria. Se asocia sobre todo al postoperatorio de cardiopatías congénitas corregidas con la operación de Fontan. Como tratamiento de base se han utilizado mucolíticos y fisioterapia respiratoria y en los casos más graves la extracción de los moldes por broncoscopia así como la nebulización de urocinasa o activador tisular del plasminógeno recombinante (rt-PA). Se presenta el caso de un varón con atresia pulmonar con tabique íntegro y ventrículo derecho hipoplásico operado a los 7 años con una intervención de Fontan, que a los 12 años presentó episodios frecuentes de tos, disnea y desaturación en los cuales el paciente expectoraba moldes bronquiales de aspecto blanquecino. Se realizó una conversión a Fontan extracardíaca para mejorar la clínica y hemodinámica, presentando en el postoperatorio frecuentes episodios de obstrucción respiratoria por moldes bronquiales que requirieron extracción mediante broncoscopia y mejoraron con la administración de urocinasa nebulizada

Plastic bronchitis is a rare complication in which bronchial casts of lymphatic origin develop in the tracheobronchial tree and cause airway obstruction. The main feature is expectoration of bronchial casts. This condition usually occurs in the postoperative period after the Fontan operation for congenital heart disease. Treatment modalities include mucolytics and chest physiotherapy, and in the most severe cases bronchoscopy to remove the casts and aerosolized urokinase or r-TPA. We describe a 12-year-old boy with pulmonary atresia with intact ventricular septum and severely hypoplastic right ventricle who underwent a modified Fontan operation at the age of 7 years. At the age of 12 years the patient started to present frequent episodes of coughing, dyspnea, and desaturation followed by difficult emission of white bronchial casts. An extracardiac Fontan conversion was performed to improve hemodynamics and symptoms. During the postoperative period frequent episodes of airway obstruction required bronchoscopy and finally improved with administration of aerosolized urokinase

Preventive-comprehensive assessment (PCA): a new screening method for subclinical cognitive problems.

Physicians often need to be able to assess cognition in a simple way, particularly for screening of subclinical processes in aged people. This paper describes a new, quick test battery called preventive-comprehensive assessment (PCA). It consists of six various testing items (repetition of three words, back-spelling of the word "sport", problem searching ina complex picture, recall of the three words, three progressive colored matrices, clock drawing test), evaluating language, recent memory, executive function, judgment and thinking capacities. The total scores. compared to the normal performance of different age groups, allow us to distinguish between normal and pathological status. If abnormal results are recorded, we need to perform further and deeper neuropsychological investigations, in order to obtain the possibly exact diagnosis. The PCA battery is sensitive and reliable; it can be used in itself, or as a part of a more complex battery of an intense comprehensive evaluation (ICE), carried out in population screening aimed at promoting healthy and active aging.

The chronically ill elderly patients discharged from the hospital: interim report from a controlled study of home care attendance.

This controlled study evaluated various outcomes in a group of 127 chronically ill and frail elderly patients when discharged from a hospital ward, and cared at home, enrolled during 12 months starting from September 2001. The observation of patients is programmed to be of two years from enrollment, with a scheduled program of follow up at baseline,6, 12, and 24 months. Patients (of both sexes) were randomly assigned to one of two groups: (i) Control group (61 patients: mean age 85.2 years) having a usual home care program of assistance, guaranteed by the Social Health Care Service. (ii) Intervention group (66 patients: mean age 83.2 years), for whom the program of care mentioned above was integrated providing collaboration of a home care attendant. This was a lay-working person who has attended a specializing course about care of the elderly and of the very frail. The home attendance was provided for 4-10 hrs daily, according to a program established by a Geriatric Evaluation Unit. The team evaluated the patients at their home,deciding the amount of hours of attendance in the presence of the caregiver. The financial cost of the additional home care attendance was sponsored, totally or in part, according to the financial situation of the patient's family, by the "Fondazione del Monte di Bologna e di Ravenna". This report takes into consideration only few preliminary data, coming from the first 6 months of observation of all the patients included. The two groups were homogeneous when compared statistically at baseline. Data coming form laboratory and biological analysis will be available only at the end of the project as they are recorded in a blind methodology. A lower degree of mortality and of dropouts in the intervention group was observed,compared to the control group, already at 6 months. These outcomes can be considered favorable, and perhaps can be related to the various benefits gained by additional program of home attendance.

Biventricular repair approach in ducto-dependent neonates with hypoplastic but morphologically normal left ventricle.

OBJECTIVES: Increased afterload and multilevel LV obstruction is constant. We assumed that restoration of normal loading conditions by relief of LV obstructions promotes its growth, provided that part of the cardiac output was preoperatively supported by the LV, whatever the echocardiographic indexes. BACKGROUND: Whether to perform uni- or biventricular repair in ducto dependent neonates with hypoplastic but morphologically normal LV (hypoplastic left heart syndrome classes II & III) remains unanswered. Echocardiographic criteria have been proposed for surgical decision. METHODS: Twenty ducto dependent neonates presented with this anomaly. All had aortic coarctation associated to multilevel LV obstruction. Preoperative echocardiographic assessment showed: mean EDLW of 12.4 +/- 3.03 ml/m2 and mean Rhodes score of -1.73 +/-0.8. Surgery consisted in relief of LV outflow tract obstruction by coarctation repair in all associated to aortic commissurotomy in one and ASD closure in 2. RESULTS: There were 3 early and 2 late deaths. Failure of biventricular repair and LV growth was obvious in patients with severe anatomic mitral stenosis. The other demonstrated growth of the left heart. At hospital discharge the EDLVV was 19.4+/-3.12 ml/m2 (p = 0.0001) and the Rhodes score was -0.38+/-1.01 (p = 0.0003). Actuarial survival and freedom from reoperation rates at 5 years were 72.5% and 46%, respectively. CONCLUSIONS: Biventricular repair can be proposed to ducto dependent neonates with hypoplastic but morphologically normal LV provided that all anatomical causes of LV obstruction can be relieved. Secondary growth of the left heart then occurs; however, the reoperation rate is high.

Surgical treatment of subaortic stenosis: a seventeen-year experience.

OBJECTIVE: The aim of the study was to analyze the long-term results of subaortic stenosis relief and the risk factors associated with recurrence and reoperation. METHODS: One hundred sixty patients with subaortic stenosis underwent biventricular repair. Before the operation the mean left ventricle-aorta gradient was 80 +/- 35 mm Hg, 57 patients had aortic regurgitation, and 34 were in New York Heart Association functional class III or IV. Median age at repair was 10 years. For discrete subaortic stenosis (n = 120), 39 patients underwent isolated membranectomy, 67 underwent membranectomy with associated septal myotomy, and 14 underwent septal myectomy. Tunnel subaortic stenosis (n = 34) was treated by myotomy in 10 cases, myectomy in 12, septoplasty in 7, Konno procedure in 3, and apical conduit in 2. Aortic valve replacement was performed in 6 cases, mitral valve replacement in 2 cases, and mitral valvuloplasty in 4 cases. RESULTS: There were 5 early (3.1%) and 4 late (4.4%) deaths. Within 3.6 +/- 3.3 years a recurrent gradient greater than 30 mm Hg was found in 42 patients (27%), 20 of whom had 26 reoperations. According to multivariable Cox regression analysis survival was influenced by hypoplastic aortic anulus (P =.01) and mitral stenosis (P =.048); recurrence and reoperation were influenced by coarctation and immediate postoperative left ventricular outflow tract gradients. At a median follow-up of 13.3 years, mean left ventricle-aorta gradient was 20 +/- 13 mm Hg. Relief of the subaortic stenosis improved the degree of aortic regurgitation in 86% of patients with preoperative aortic regurgitation. Actuarial survival and freedom from reoperation rates at 15 years were 94% +/- 1.3% and 85% +/- 6%, respectively. CONCLUSION: Although surgical treatment provides good results, recurrence and reoperation are significantly influenced by previous coarctation repair and by the quality of initial relief of subaortic stenosis.

Two-stage correction of transposition of great arteries with complete atrioventricular canal.

We report a successful two-stage anatomic correction of D-transposition of the great arteries associated with complete atrioventricular canal without pulmonary obstruction, combining an arterial switch procedure and a two-patch repair for complete atrioventricular canal, after pulmonary artery banding in the neonatal period.

Biventricular repair of conotruncal anomalies associated with aortic arch obstruction: 103 patients.

BACKGROUND: Biventricular repair of conotruncal anomalies associated with aortic arch obstruction is a complex surgical procedure that combines a cardiac repair and a aortic arch reconstruction. METHODS AND RESULTS: From January 1984 to April 1996, such a repair was performed in 103 patients. The conotruncal anomalies included: 15 transpositions of the great arteries (TGAs) with intact ventricular septum, 44 TGAs with ventricular septal defect, 32 double outlet right ventricle with subpulmonary ventricular septal defect, 10 truncus arteriosus, one double outlet left ventricle, and one tetralogy of Fallot. The arch obstruction included 88 coarctation and 15 interrupted aortic arch. One-stage repair has been the favored technique since 1990 and was performed in 58 neonates, including 38 TGAs or double outlet right ventricle and ventricular septal defect, 10 TGAs with intact ventricular septum, and all of the 10 truncus arteriosus. The cardiac repair included 89 arterial switch operations, 2 Kawashima rerouting, 10 truncus arteriosus repairs, and one double-outlet left ventricle repair and one tetralogy of Fallot repair. The aortic arch was reconstructed by direct anastomosis in 85 patients, with a Gore-Tex conduit in three patients and more recently by an ascending aortic patch augmentation in 15 patients. The hospital mortality was 12% (7 of 58) for the one-stage repair and 20% (9 of 45) for the two-stage repair. There were six late deaths. Reoperations or angioplasties were mandatory for 12 right ventricle outflow tract obstructions after arterial switch, involving 10 patients with double outlet right ventricle (P=.02), 10 recurrent arch obstruction, and six miscellaneous lesions. CONCLUSIONS: One-stage biventricular repair of conotruncal anomalies associated with aortic arch obstruction can be achieved in selected patients with an 83% survival rate at 7 years.

Extracardiac Fontan operation for complex cardiac anomalies: seven years' experience.

METHODS: Between 1988 and 1995, 60 patients with complex cardiac anomalies underwent a total extracardiac cavopulmonary connection, a combination of a bidirectional cavopulmonary anastomosis with an extracardiac conduit interposition between the inferior vena cava and pulmonary arteries, except in one patient in whom direct anastomosis was possible. In 40 patients the total extracardiac cavopulmonary connection followed preliminary bidirectional cavopulmonary anastomosis, associated with a modified Damus-Kaye-Stansel anastomosis in 16. The conduits were constructed of Dacron fabric (n = 34), homografts (n = 3), and polytetrafluoroethylene (n = 22). RESULTS: Total early failure rate was 15% (n = 9). Six patients died, and three more had conduit takedown owing to pulmonary artery stenosis and hypoplasia (n = 2) and severe atrioventricular valve regurgitation (n = 1). Two other patients required anastomosis revision owing to stricture. In a mean follow-up of 48 months (6 to 86 months) there were no late deaths (actuarial 5-year survival 88% +/- 4%); 52 of 54 patients are in New York Heart Association class I or II. Two patients required pulmonary artery balloon dilation or stent implantation, or both, after total extracardiac cavopulmonary connection. Late tachyarrhythmias were detected in four of 54 patients: two had sick sinus syndrome with flutter necessitating a pacemaker implantation and two had recurrent flutter (actuarial 5-year arrhythmia-free rate 92% +/- 4%). Conduit patency was evaluated by serial magnetic resonance imaging studies. Preliminary data showed a 17.8% +/- 7.6% mean reduction in conduit internal diameter during the first 6 months after total extracardiac cavopulmonary connection, with no progression over the next 5 years. CONCLUSION: These results demonstrate that the total extracardiac cavopulmonary connection provides good early and midterm results and may reduce the prevalence of late arrhythmias in patients undergoing the Fontan operation.

Surgery for congenital mitral valve disease in the first year of life.

Between 1980 and 1993, 20 patients less than 1 year of age underwent operations for congenital mitral valve disease. Ten patients had congenital mitral incompetence and 10 had congenital mitral stenosis. Mean age was 6.6 +/- 3.4 months and mean weight was 5.6 +/- 1.5 kg. Atrioventricular canal defects, univentricular heart, class III/IV hypoplastic left heart syndrome, discordant atrioventricular and ventriculoarterial connections, and acquired mitral valve disease were excluded. Indications for operation were intractable heart failure or severe pulmonary hypertension, or both. Associated lesions, present in 90% of the patients, had been corrected by a previous operation in seven. In congenital mitral incompetence there was normal leaflet motion (n = 3), leaflet prolapse (n = 2), and restricted leaflet motion (n = 5). In congenital mitral stenosis anatomic abnormalities were parachute mitral valve (n = 4), typical mitral stenosis (n = 3), hammock mitral valve (n = 2), and supramitral ring (n = 1). Mitral valve repair was initially performed in 19 patients and valve replacement in one with hammock valve. Concurrent repair of associated lesions was performed in 12 patients. The operative mortality rate was zero. There were six early reoperations in five patients for mitral valve replacement (n = 4), a second repair (n = 1), and prosthetic valve thrombectomy (n = 1). One late death occurred 9 months after valve replacement. Late reoperations for mitral valve replacement (n = 2), aortic valve replacement (n = 1), mitral valve repair (n = 2), subaortic stenosis resection (n = 1), and second mitral valve replacement (n = 1) were performed in five patients. Actuarial freedom from reoperation is 58.0% +/- 11.3% (70% confidence limits 46.9% to 68.9%) at 7 years. After a mean follow-up of 67.6 +/- 42.8 months, 94% of living patients are in New York Heart Association class I. Doppler echocardiographic studies among the 13 patients with a native mitral valve show mitral incompetence of greater than moderate degree in one patient and no significant residual mitral stenosis. Overall, six patients have mitral prosthetic valves with a mean transprosthetic gradient of 6.2 +/- 3.7 mm Hg. These results show that surgical treatment for congenital mitral valve disease in the first year of life can be performed with low mortality. Valve repair is a realistic goal in about 70% of patients and possibly more with increased experience. Reoperation rate is still high and is related to complexity of mitral lesions and associated anomalies, but late functional results are encouraging.

Staged Fontan operation for complex cardiac anomalies with subaortic obstruction.

Ventricular hypertrophy is a recognized risk factor for the Fontan operation in cases of complex cardiac anomalies with unrestricted pulmonary blood flow and subaortic obstruction. Between 1986 and 1991 we have treated 23 such patients with a new type of palliation combining a main pulmonary artery-ascending aorta anastomosis with a bidirectional cavopulmonary anastomosis. Their ages averaged 57 +/- 36 months (7 to 155 months). Nine patients had (S,L,L) single ventricule with transposition and the other 14 had more complex cardiac anomalies unsuitable for biventricular repair. Subaortic obstruction was defined by a hemodynamic criterion (n = 6) or by a morphologic criterion (n = 17). There were five hospital deaths (21.7%): All three patients older than 7 years of age died (p = not significant). Follow-up averaged 28 +/- 21 months (range 1 to 58 months). Among the 18 hospital survivors, the proportions surviving 1 and 5 years after the operation were 78% and 63%, respectively. Control cardiac catheterization in 11 patients showed no or trivial subaortic gradient, a mean cavopulmonary pressure of 10 +/- 3 mm Hg, and a mean arterial oxygen saturation of 83%. Nine patients underwent secondary Fontan repair in our institution 21 +/- 4 months after palliation, without deaths. Another patient died elsewhere, 3.7 years after palliation, as a result of hemorrhage at sternal reentry during attempted Fontan operation (overall mortality at repair, 10%). The other eight patients are awaiting Fontan operation. This staged approach reduces both pressure and volume ventricular load and provides adequate oxygenation before the Fontan operation. In our experience, it resulted in reduced mortality at definitive repair.