Mild Atrioventricular Valve Regurgitation Research Articles

Late outcomes of Fontan operation from the EUROFONTAN experience

Abstract Background Fifty years after the first Fontan operation, the persisting challenges and evolving landscape of post-Fontan complications are explored in the EUROFONTAN experience. Aim of the study is the evaluation of long term outcomes of a large cohort of Fontan patients, focusing on age at Fontan and onset of Fontan-associated liver disease (FALD). Methods A retrospective clinical study across 21 european congenital cardiac centers across Europe. Clinical and operative data of patients who underwent Fontan operation between January 1990 and December 2022 were gathered through a RedCap online database. Results 2606 patients were included in this analysis (M/F: 59%/41%, median age and median weight at Fontan of 4.3 y.o (IQR 3.3-6.4), and 16kg (IQR 14-20), respectively. Most frequent cardiac anomaly were tricuspid atresia and pulmonary atresia with intact ventricular septum (781, 30%). Ventricular morphology was left in 1379 patients (53%), and right in 1029 patients (40%). Extra cardiac conduit (ECC) was performed in the majority of patients (2335, 90%), with a median conduit size of 20mm (IQR 18-20). Early post-operative complications were present in 52% (1340), with an early reintervention in 349 patients (249 surgical , 99 catheter driven) At a median follow-up median of 10 years (IQR 4-15), there were 79 late deaths (3%). Adverse events occurred in 49% (1288 , non-surgical in 962 , surgical in 249). The clinical status was stage A in 60% (1288 patients), while only a minority (1.3%) was in status D. Systemic Ventricular function was normal in 1898 patients (73%), while absent or mild Atrioventricular Valve regurgitation was presnet in 86% (1816). Of note, FALD was detected in 388 (15 %) , and sinus rhythm was present in 99%. Statistical analysis shows that late death and adverse events are significantly associated right ventricular morphology of the systemic ventricle (p-value 0.019). Of note, when compared to lateral tunnel, TCPC extracardiac conduit demonstrated lower late mortality (2.3% vs 8.2%, p-value <0.001) and complication rates (60% vs 48%, p-value 0.001). Analyzing age at Fontan, a linear relationship exists between age and late mortality, whereby mortality increases with advancing age (graph 1). However, concerning the development of late adverse events, the risk decreases with age at Fontan up to 7 years; beyond 7 years, the risk increases with advancing age (graph 2). The analysis demonstrated growing FALD-prevalence with increasing time from Fontan completion (significant especially after 10 years). Conclusion current long term outcomes of Fontan operation are highly satisfactory. However, the longer survival is associated to an increasing incidence of late term extracardiac complications (namely FALD) which are more evident if Fontan is performed at older age.Data of Fontan PatientsCorrelation between age at Fontan and AE

Echocardiographic values and prevalence of cardiac abnormalities in clinically healthy adult Borzoi dogs.

To develop breed-specific echocardiographic values for normal Borzoi and to report the prevalence of structural cardiac abnormalities. 146 clinically healthy, adult Borzoi dogs. Cardiac auscultation and standard echocardiograms were performed. Longitudinal follow-up was described in a subset of dogs (n = 25). Most Borzoi were structurally normal (119/146, 81.5%), with breed-specific echocardiographic values generated independently for each sex, as females weighed significantly less than males (30.4 ± 3.8 kg vs 38.3 ± 4.1 kg, respectively; P < .001), and a significant impact of sex was found on most measurements. Physiologic heart murmurs were identified in 64/119 (53.8%) normal dogs. Thirty-six (30.2%) structurally normal dogs had trace or mild mitral regurgitation, and 43 (36.1%) had trace or mild tricuspid regurgitation. Structural cardiac disease was identified in 21 dogs (14.4%), including 9 dogs (6.2%) with dilated cardiomyopathy (DCM), 9 dogs (6.2%) with stage B1 myxomatous mitral valve disease (MMVD), and 3 (2.1%) dogs with congenital abnormalities. Seven dogs (4.8%) had equivocal abnormalities. During follow-up, new dogs were diagnosed with occult DCM (n = 3), equivocal DCM (1), and stage B1 MMVD (2). Two dogs originally diagnosed with DCM (1 occult and 1 equivocal) normalized after diet change. Borzoi dogs commonly have physiologic heart murmurs and mild atrioventricular valve regurgitation. Both DCM and MMVD were identified at similar frequencies in healthy Borzoi, although dogs with MMVD all had normal heart sizes. Echocardiographic screening for DCM in Borzoi should be considered, with breed-specific echocardiographic values now available for improved diagnostic confidence.

A multicenter, retrospective study of cardiac disease in Borzoi dogs.

Borzoi are large, relatively uncommon sighthounds anecdotally reported to suffer from sudden death. This multicenter retrospective cohort study aimed to describe the sample of Borzoi presenting to veterinary cardiologists for evaluation, with records searched from 14 centers across a study period of up to 20 years. The study sample was comprised of 152 client-owned Borzoi, with dogs most commonly presenting for pre-breed screening in 87/152 (52%), followed by evaluation of an arrhythmia in 28/152 (18%). Of the 131/152 (86%) dogs that had an echocardiogram performed, 85/131 (65%) were structurally normal, with 40/85 (47%) structurally normal dogs having trace or mild atrioventricular valve regurgitation. Tricuspid valve dysplasia was the most commonly diagnosed congenital cardiac disease (n = 6). Myxomatous mitral valve disease (n = 12) and dilated cardiomyopathy (n = 13) were diagnosed at similar frequencies, though 92% of valve disease cases were mild. Only 48/152 (32%) Borzoi had a diagnostic electrocardiogram (ECG) and/or a Holter monitor for arrhythmia screening. Despite this, ventricular arrhythmias were identified during the entirety of the available cardiac evaluation including diagnostic ECG, contemporaneous ECG monitoring during the echocardiogram, and/or Holter monitor in 25/131 (19%) dogs in which an echocardiographic diagnosis was available. Of these 25 Borzoi, 76% had minimal or no structural cardiac disease identified, and five had a family history of sudden death. A sudden death outcome was reported in 3/55 (5%) Borzoi with long-term outcome data available. In conclusion, Borzoi commonly have trace or mild atrioventricular valve insufficiencies, and may develop ventricular arrhythmias and dilated cardiomyopathy.

Surgical reintervention on the neo-aorta after the Norwood operation.

We sought to identify the prevalence of surgical reintervention on the neo-aorta after Norwood procedure and its impact on long-term outcomes. We reviewed the medical records of all patients who underwent Norwood procedure. The impacts of surgical neoaortic reintervention on outcomes were analysed in each stage of palliation. A total of 335 patients were included in this study. Thirty patients underwent surgical reintervention on the neo-aorta after Norwood procedure. The timing of initial reintervention was before stage II in 13 patients, at stage II in 7, between stage II and stage III in 5, at stage III in 3 and after stage III in 2. A reintervention before stage II was significantly associated with mortality (HR 14.4, 95% confidence interval 6.00-34.6, P < 0.001). In patients who underwent stage II (n = 251), reintervention had no significant impact on mortality. In patients who underwent stage III (n = 188), the previous reintervention was significantly associated with higher mean pulmonary pressure (P = 0.05) and a higher rate of reduced ventricular function (P = 0.002). Greater than mild atrioventricular valve regurgitation was significantly associated with the development of a neoaortic arch stenosis after stage II (P = 0.03). Surgical reinterventions on the neo-aorta were required in each inter-stage phase. A surgical neoaortic reintervention was not related to increased mortality after stage II but significantly associated with a higher rate of reduced ventricular function and elevated mean pulmonary artery pressure.

Outcomes of single ventricle palliation in infants with heterotaxy syndrome.

Heterotaxy is a known risk factor for morbidity and mortality in single ventricle palliation. In this study, we examined our experience with this challenging group of patients. Records of patients born between 2001 and 2019 with heterotaxy, who needed staged single ventricle palliation were retrospectively analysed. A total of 53 patients were included in this study. Thirty-five (66%) patients had a right ventricular dominance, common atrioventricular septal defect was present in 37 (70%) patients. Anomalous pulmonary venous drainage was present in 29 (55%) patients. Forty-six (86%) patients underwent first-stage palliation. Forty-one (77.3%) patients received a bidirectional cavopulmonary connection. Thirty-one (58%) patients received total cavopulmonary connection (TCPC). Overall survival rate was 92% at 1 month, 74% at 1 year and 68% at 10 years. Twelve (22.6%) patients died before second palliation stage. Four (10%) patients died before TCPC. No patient died after TCPC. Independent risk factors for mortality in the multivariate COX regression were a presence of restrictive pulmonary blood flow (HR 3.23; 95% CI 1.02-10.2; P = 0.05) and greater than mild atrioventricular valve regurgitation (HR 3.57; 95% CI 1.27-10.0; P = 0.02). Mortality and morbidity in patients with single ventricle and heterotaxy are high. Restrictive pulmonary blood flow needing early modulation and greater than mild atrioventricular valve regurgitation at presentation are independent risk factors for mortality. Total anomalous pulmonary venous connection was not identified as a risk factor in the current era.

Initial fetal to initial postnatal echocardiogram in uncomplicated atrioventricular septal defects: Do significant changes occur?

Yield of serial echocardiography in fetuses with atrioventricular septal defects (fAVSD) has not been well defined. The goal of this study was to document if any major changes occurred from initial fetal to initial postnatal echocardiogram in uncomplicated fAVSD. fAVSD were excluded if initial fetal scan documented complex CHD or any concerns. Changes in ventricular function, valvular regurgitation, or diagnosis between first fetal and first postnatal echocardiogram were recorded. Fifty-seven fAVSD met criteria. Ninety-six fetal echocardiograms were done in 57 patients. Initial fetal scan was performed at 24.3±3.7weeks of estimated gestational age. All fAVSD had normal function, 38 had no atrioventricular valve regurgitation (AVVR), and 19 had mild AVVR. First postnatal echocardiogram was performed at 6.3±15.3days. Fifty-six patients had normal function, 1 patient had mild dysfunction, 16 patients had no AVVR, 36 had mild AVVR, and 5 had moderate AVVR. Three patients (5%) had an improvement in AVVR by one degree, 27 patients (47%) had no change in AVVR, 24 patients (42%) had an increase in AVVR by one degree, and 3 patients (5%) had an increase in AVVR by two degrees. There was no major missed anatomical diagnosis from first prenatal to first postnatal echocardiogram. In fAVSD that had no concerns on their initial fetal echocardiogram, the majority of patients had no major changes noted between their initial fetal echocardiogram and their first postnatal echocardiogram. Repeat fetal echocardiograms may not necessarily be needed in this cohort of patients.

Augmentation of Pulmonary Arterial Growth in Single Ventricle Patients by Interim Selective Shunts

Stenosis or diffuse hypoplasia of central pulmonary arteries (PA) is common in patients with single ventricle physiology, often requiring surgical patching. Such repairs are prone to failure, particularly with low pressure venous flow (bidirectional cavopulmonary connection or Fontan). We describe our experience of disconnection of central PA and selective systemic-PA shunt to the hypoplastic vessel. Single ventricle patients (n = 12) with diffuse left pulmonary artery (LPA) hypoplasia (LPA:right pulmonary artery diameter <0.7) underwent PA disconnection (ligation clip) and selective arterial shunt to the LPA. Patients with ≤mild atrioventricular valve regurgitation, and no more than mild systolic dysfunction on echocardiogram were considered. Following systemic-LPA shunt, patients were reassessed by cardiac catheterization prior to further surgery, with follow-up catheterization later performed and description of changes observed. Increased volume loading was well tolerated with no greater than mild atrioventricular valve regurgitation and preserved systolic function (normal or mildly reduced). Selective arterial shunting increased the caliber of the LPA from 4.1 mm (1.2-5.6) to 6.5 mm (1.7-11.9) and this increase was preserved post-Fontan (6.7 mm [1.3-8.0]) (median [range]). Ventricular end diastolic pressure increased with arterial shunting but resolved after shunt takedown and Fontan completion (median +3 and -4 mm Hg respectively). Post-Fontan hospital length of stay was not prolonged (median 11 days, range 7-14). No deaths occurred. In univentricular hearts and PA hypoplasia, selective systemic-PA shunting physiologically increases the caliber of the distal vessels. In selected patients this can be done safely with maintenance of PA growth and resolution of the elevated end diastolic pressure with Fontan completion.

P267 Dextrocardia with single ventricle and symptomatic erythrocytosis: a case report

Abstract Introduction Dextrocardia is a rare cardiac anomaly which refers to a right-sided location of the heart within the thoracic cavity. Dextrocardia may be associated with other complex cardiac malformations which could result in cyanosis. We reported a case of cyanotic dextrocardia with symptomatic erythrocytosis. Case Report A 54-year-old Asian male was admitted to hospital with the complaint of numbness and tingling of limbs for one month. There was no history of chronic cardio-cerebrovascular diseases or diabetes. On examination vital signs were as follows: blood pressure 112/73 mmHg, pulse 72 beats/min, oxygen saturation 85% on room air, temperature 37.1°C, respiratory rate 18 breaths/min. Cardiac physical examination was indicative of dextrocardia: the apex of the heart was located in the right side of the chest accompanied with a systolic murmur at the apex. Other pertinent examinations showed cyanosis of lips, flushing of face and limbs, and a normal neurological examination. Echocardiography and computed tomography (CT) demonstrated heterotaxy and dextrocardia, normal atriums, common mitral valve, lengthy leaflets of tricuspid valve, dilated single ventricle, double-inlet and outlet left ventricle, mild atrioventricular valve regurgitation (AVVR) and no pulmonary stenosis. Laboratory data showed increased hemoglobin of 19.9 g/dl and hematocrit of 58.8%, decreased oxygen partial pressure of 49.5mmHg and oxygen saturation of 81.3%. The patient was diagnosed with erythrocytosis perhaps secondary to cyanotic congenital heart disease and was given intravenous drip of normal saline to resolute the hyperviscosity status. He refused bone marrow aspiration and surgical evaluation for single-ventricle palliation. During the 16-month follow up, no other symptoms emerged except for mild numbness of limbs. Conclusion Dextrocardia with single ventricle malformation can remain symptom free for a long time until the emergence of complications such as secondary erythrocytosis. It is important to give an early diagnosis and complete evaluation, and echocardiography is an effective method to detect this anomaly. Abstract P267 Figure. The echo and CT images of this case

Hypoplastic Left Heart Syndrome Is Not a Predictor of Worse Intermediate Mortality Post Fontan

An increasing proportion of those living with single ventricle physiology have hypoplastic left heart syndrome (HLHS). Our objective was to assess the association between HLHS and outcomes post Fontan operation. All pediatric patients who underwent a Fontan procedure at the University of Alberta between 1996 and 2016 were included. Follow-up clinical data collected included early and late surgical or catheter reintervention, echocardiography, and long-term transplant-free survival. Characteristics were compared between those with and without HLHS, and the association between outcomes and HLHS were assessed. A total of 320 children (median age 3.3 years, interquartile range 2.8 to 3.9 years; 121 [43.4%] female) underwent a Fontan procedure over the course of the study. Nearly one third of subjects had HLHS (107, 33.4%). Patients with HLHS were more likely to have abnormal ventricular function (19.6% versus 7.0%, p= 0.003) and worse than mild atrioventricular valve (AVV) regurgitation (23.4 versus 9.2%, p= 0.001) preoperatively. HLHS was not predictive of in-hospital Fontan failure (odds ratio 0.82, 95% CI 0.28, 2.39), late reintervention (hazard ratio [HR] 1.08, 95% CI 0.66, 1.76), or transplant-free survival (HR 1.58, 95% CI 0.72, 3.44). Subjects with HLHS were more likely to have more than mild AVV regurgitation (31.6% versus 13.3%, p= 0.028) and abnormal ventricular function (29.8% versus 10.7%, p < 0.0001) at late follow-up. Patients with HLHS who survive to the Fontan procedure do no worse with the operation than those with other anatomy. Given worse late ventricular function and AVV regurgitation, equivalent survival may not persist throughout a patient's life course.

Outcomes of Fontan conversion for failing Fontan circulation: mid-term results.

We investigated the results of a revision of a previous Fontan connection to total cavopulmonary connection (TCPC) in patients with failing Fontan circulation. From July 1998 to April 2013, 21 patients who had failing Fontan circulation underwent revision of the previous Fontan operation to TCPC. The median age at TCPC conversion was 17.9 years (range, 4.6-38.1 years) and the median interval between initial Fontan operation and TCPC was 13.8 years (range, 2.1-25.4 years). There were 37 indications for Fontan revision in 21 patients. The indications were huge right atrium (n = 15), atrial arrhythmia (n = 8), intra-atrial thrombi (n = 6), protein-losing enteropathy (PLE) (n = 3) and more than mild atrioventricular valve regurgitation (n = 5). The previous Fontan operation was revised to extracardiac conduit replacement (n = 20) and intra-atrial lateral tunnel (n = 1). Concomitant surgery for atrial arrhythmia was performed in 8 patients. Fenestration was performed in 7 patients. The median follow-up duration was 7.1 years (range, 0.3-13.4 years). There were no operative deaths and two late deaths occurred 7.9 and 8.1 years after operation. Actuarial 5- and 10-year survival rates were 92.3 and 83.1%, respectively. Postoperative complications included bleeding (n = 3), deep sternal infection (n = 1) and prolonged pleural effusion for more than 2 weeks (n = 5). During follow-up, atrial arrhythmia recurred in 6 patients, PLE recurred in 2 patients and pleural effusion recurred in 2 patients. All patients were classified as New York Heart Association Class I (n = 15) or Class II (n = 4). Fontan conversion to TCPC in patients with failing Fontan circulation can be performed with low risk of morbidity and mortality. The procedure confers better quality of life and is functional for patients with failed Fontan circulation.

Results of Fontan operation in patients with atrioventricular valve regurgitation.

The influence of concomitant atrioventricular valve regurgitation on the results of Fontan operation remains disputable. The goal of this study was to compare early and late results of Fontan operation performed in patients with mild or moderate-to-severe atrioventricular valve regurgitation. For retrospective analysis, patients with atrioventricular valve insufficiency assessed by echocardiography were divided into three groups based on the severity of regurgitation and its repair or non-repair during Fontan operation: Group 1: unrepaired regurgitation 1-2+ (n = 33); Group 2: unrepaired regurgitation 3-4+ (n = 11); Group 3: repaired regurgitation 3-4+ (n = 35). Actuarial survival was estimated by the Kaplan-Meier method, followed by the log-rank test to compare survival curves between groups. Hospital mortality rates in Groups 1 through 3 reached 3, 27 and 14%, respectively (P = 0.015, Group 1 vs Group 2). The frequency of non-lethal complications did not significantly differ between groups. However, patients from Group 2 required significantly more intensive inotropic support, longer mechanical ventilation and had larger pleural effusion. Predicted survival after Fontan operation was the worst in Group 2 (P = 0.016, Group 2 vs Group 1). The frequency of non-lethal late complications was also the highest in Group 2 (50 vs 17 or 11%); however, the difference did not reach statistical significance (P = 0.13 and 0.069, respectively). The severity of atrioventricular valve regurgitation during the follow-up did not significantly change when compared with discharge after the repair in Group 2 or Group 3 (P = 0.19 and 0.52, respectively), and significantly increased in Group 1 (P = 0.003). However, this increase did not have clinical significance during the reported period of follow-up. If unrepaired, concomitant moderate-to-severe atrioventricular valve regurgitation significantly worsens the results of the Fontan procedure. Longer observation is needed to define the strategy in patients with mild atrioventricular valve regurgitation that remained unrepaired during Fontan operation.

Increased common atrioventricular valve tenting is a risk factor for progression to severe regurgitation in patients with a single ventricle with unbalanced atrioventricular septal defect

Significant atrioventricular valve regurgitation (AVVR) increases mortality in patients with unbalanced atrioventricular septal defects (uAVSDs) and a single ventricle. We tested the hypothesis that abnormal leaflet tethering is associated with progressive AVVR in patients with a single ventricle with uAVSD. We retrospectively reviewed the initial presentation and prebidirectional cavopulmonary anastamosis echocardiograms of 46 consecutive patients with uAVSD with single ventricle palliation. AVVR was graded as moderate to severe if the sum of vena contracta width to dominant valve annulus ratio was ≥ 0.33. We measured tenting height, annular to leaflet angle and annular diameter, indexed to patient size where appropriate. Multivariate analysis of variables to predict progressive AVVR was performed. At follow-up of 3.3 ± 2.4 years, 24 patients had mild AVVR (Group A) and 22 had moderate to severe AVVR. Overall mortality was 6%, whereas 10 had valve repair/replacement surgery. Of 22 patients with severe AVVR at follow-up, 9 had severe AVVR at initial presentation (Group B), whereas 13 had mild AVVR at presentation but developed severe AVVR at their prebidirectional cavopulmonary anastamosis echocardiogram (Group C). Group A patients had a smaller tenting height at initial presentation compared with patients in Group B and Group C, and also had early progressive reduction of indexed tenting height (P < .01). An absolute tenting height >6 mm (odds ratio, 6.6; 95% confidence interval, 1.1-39.0; P = .03) at the initial echocardiogram was identified as an independent predictor of subsequent severe AVVR. Early leaflet tethering is predictive of subsequent AVVR in patients with a single ventricle with uAVSD. Patients with competent AVV had progressive reduction in the degree of leaflet tethering, whereas patients with AVVR did not. This may represent an important adaptive process to maintain valve competency in uAVSD.

Surgical Treatment of Infective Valve Endocarditis in Children with Congenital Heart Disease

This study assesses surgical procedures, operative outcome, and early and intermediate-term results of infective valve endocarditis in children with congenital heart disease. Seven consecutive children (five females, two males; mean age, 10.8 years) who underwent surgery for infective valve endocarditis between 2006 and 2010 were included in the study. The aortic and mitral valves were affected in two and tricuspid in five patients. Indications for operation included cardiac failure due to atrioventricular septal rupture, severe tricuspid valve insufficiency, and septic embolization in one, moderate valvular dysfunction with vegetations in three (two tricuspid, one mitral), and severe valvular dysfunction with vegetations in the other three patients (two tricuspid, one mitral). The pathological microorganism was identified in five patients. Tricuspid valve repair was performed with ventricular septal defect (VSD) closure in five patients. Two patients required mitral valve repair including one with additional aortic valve replacement. There were no operative deaths. Actuarial freedom from recurrent infection at one and three years was 100%. Early echocardiographic follow-up showed four patients to have mild atrioventricular valve regurgitation (three tricuspid and one mitral) and three had no valvular regurgitation. No leakage from the VSD closure or any valvular stenosis was detected postoperatively. Mitral and tricuspid valve repairs can be performed with low morbidity/mortality rates and satisfactory intermediate-term results in children with infective valve endocarditis.

Risk factors for failed staged palliation after bidirectional Glenn in infants, who have undergone stage one palliation☆

The bidirectional Glenn procedure (BDG) is a routine intermediary step in single-ventricle palliation. In this study, we examined risk factors for death or transplant and failure to reach Fontan completion after BDG in patients, who had previously undergone stage one palliation (S1P). All patients at our institution, who underwent BDG following S1P between 2002 and 2009 (n=194), were included in the analysis. Transplant-free survival through 18 months post BDG was 91%. Univariable competing risk analyses showed atrioventricular valve regurgitation (AVVR) >mild, age ≤ 3 months at BDG, ventricular dysfunction >mild, and prolonged hospital stay after S1P to be associated with increased risk of death or orthotopic heart transplant. Multivariable competing risk analysis through 5 years of follow-up showed >mild AVVR (hazard ratio (HR) 7.5, 95% confidence interval (CI) 3.0-18.8), prolonged hospitalization after S1P (HR 4.5, 95% CI 1.8-11.5), and age ≤ 3 months at BDG (HR 6.8, 95% CI 2.3-20.0) to be independent risk factors for death or transplant. Concomitantly, > mild AVVR and age ≤ 3 months were independently associated with an overall decreased rate of Fontan completion. Pre-BDG AVVR, age ≤ 3 months at time of BDG, and prolonged hospitalization after S1P are independently associated with decreased successful progression of staged palliation in midterm follow-up after BDG.

Neonatal Mitral and Tricuspid Valve Repair for In Utero Papillary Muscle Rupture

Papillary muscle rupture in the fetus and neonate is a rare event that leads to severe mitral or tricuspid insufficiency and is associated with high perinatal mortality. We undertook surgical repair of this lesion in the neonatal period and report on our midterm results. Three neonates with tricuspid insufficiency and 1 infant with mitral insufficiency, all due to papillary muscle or chordal rupture, underwent surgical repair with artificial chordal replacement and a modification of the de Vega annuloplasty technique that allowed external adjustment of the annulus size under transesophageal echocardiographic guidance after separation from cardiopulmonary bypass. All patients recovered well from the operation. There have been no late deaths and no valve-related complications. On discharge, all 3 patients had evidence of trace to mild atrioventricular valve regurgitation. At a median follow-up of 33 months (range, 7 to 50; 123 patient-months), all 4 patients are growing normally. Three patients have had no change in the degree of tricuspid or mitral regurgitation. One patient required reoperation at 54 months postoperatively for acute mitral insufficiency secondary to separation of an artificial chorda from the ventricular wall. Surgical repair of critical neonatal tricuspid and mitral insufficiency associated with papillary muscle or chordal rupture is feasible and can result in good early and midterm results. Our modification of the De Vega annuloplasty technique with the ability to externally adjust the size of the annulus under echocardiographic guidance may improve the accuracy of the repair in the neonate.

Utilization of Double-Orifice Valve Plasty in Correction of Atrioventricular Septal Defect

Atrioventricular valve regurgitation represents the principal indication for reoperation after repair of atrioventricular septal defect. Deciding how to correct atrioventricular valve regurgitation is challenging in some cases because of the complexity of the anatomic features. This report deals with our surgical experience in using a double-orifice valve plasty technique in cases with atrioventricular septal defect. From August 2002 to August 2004, 8 patients underwent double-orifice valve plasty in surgical correction of atrioventricular septal defect. Anatomic types were partial (6 patients), intermediate (1 patient), and complete (1 patient). After the mitral cleft was closed, moderate to severe atrioventricular valve regurgitation was still present in these patients. Double-orifice valve plasty was used in the mitral valve in 7 patients and in the tricuspid valve in 1. No hospital deaths or postoperative morbidity occurred. The follow-up ranged from 6 months to 30 months (median, 14.4 months). No or trivial atrioventricular valve regurgitation was found in 6 patients and mild atrioventricular valve regurgitation was present in 2. Double-orifice valve plasty is an easy and effective additional procedure for children and for adult patients who have moderate or severe atrioventricular valve regurgitation after repair of atrioventricular septal defect.

Repair of atrioventricular valve regurgitation in the modified Fontan operation1

To evaluate the prevalence, impact and outcome of repair of atrioventricular valve regurgitation (AVVR) in Fontan patients. We retrospectively reviewed 340 Fontan patients from 1986 to 2001. Twelve patients with valve closure or replacements were excluded. AVVR was graded by transthoracic echocardiography. Patients were divided into group O (no AVVR), group A (1+ to 2+) and group B (3+ to 4+). AVVR was present in 129 (39.3%) patients. Repair was carried out in 37 (11.3%) with no difference in mortality as compared to no repair (18.9 vs 10.9%, P =0.16). Mean follow-up was 44 months (1-197 months) with 14 patients lost to follow-up. No valve repair was carried out in group O (192 patients) and no clinical change in AVVR was observed (pre-op 0.00, post-op 0.78). In group A (85 patients) 6 patients had valve repair without significant change in the degree of AVVR after valve repair (pre-op 1.8, post-op 2.2, P = 0.18). In group B (44 patients) 31 had valve repair and a significant reduction was observed (pre-op 3.28, post-op 2.44, P < 0.001). A similar improvement was also observed when the valve was not repaired (pre-op 3.25, post-op 2.44, P = 0.003). Survival at 10 years was comparable (83% repaired, 89% not repaired, P = 0.165). There was no difference in the incidence of long-term complications (26% repaired, 29.7% no repair, P = 0.64). Trivial to mild AVVR remains stable and their repair during the Fontan operation provides no additional benefits. Valve repair in patients with moderate to severe AVVR improved the regurgitation with comparable operative mortality and long-term outcome; however, similar benefits could be achieved without repair of the atrioventricular valve. We should not deny patients with similar AVVR the Fontan operation.

Surgical Repair of Complete Atrioventricular Septal Defect

Between January 1988 and March 1996, 40 patients underwent repair of complete atrioventricular septal defect with a two-patch technique and routine atrioventricular valve cleft closure. The mean age of the patients was 10.8 ± 6.9 months and the mean weight was 6.6 ± 2.6 kg. Twenty-three had Down's syndrome and 13 had coexisting cardiac anomalies. Preoperative angiography and echocardiography revealed mild atrioventricular valve regurgitation in 22 patients, moderate regurgitation in 16, and severe regurgitation in the other 2. The mortality was 12.5% (4 early and 1 late deaths). The major cause of death was pulmonary hypertensive crisis. Reoperation was necessary in 3 patients; 2 had atrioventricular valve annuloplasty and one had prosthetic valve replacement. All 3 survived reoperation. Echocardiography at a mean of 32 ± 20 months postoperatively showed mild left atrioventricular valve regurgitation in 32 patients and moderate regurgitation in 3. Management of postoperative pulmonary hypertensive crisis and repair of complete atrioventricular septal defect before the development of high pulmonary vascular resistance may reduce the mortality of this surgical procedure.

Factors influencing survival of patients with heterotaxy syndrome undergoing the Fontan procedure

Objectives. This study was undertaken to determine those factors that may influence survival in patients with heterotaxy syndrome undergoing the Fontan procedure.Background. The Fontan procedure remains the preferred palliative procedure for patients with heterotaxy syndrome. Although the mortality rate has improved for patients without this syndrome undergoing the Fontan procedure, it remains high for patients with heterotaxy syndrome.Methods. The medical records of 20 consecutive pediatric patients with asplenia (n = 12) and polysplenia (n = 8) who underwent the Fontan procedure between January 1, 1986 and December 31, 1990 were reviewed. Anatomic and hemodynamic data were collected, as well as data on types of surgical palliative procedures and on outcome of the Fontan procedure.Results. There were two early and two late deaths for a total mortality rate of 20% in the patients with heterotaxy syndrome, as compared with 8.5% for the patients without this syndrome who underwent the Fontan procedure during the same time period. Factors that significantly increased the risk of the Fontan procedure in these patients were 1) preoperative findings of greater than mild atrioventricular valve regurgitation, b) hypoplastic pulmonary arteries, and c) mean pulmonary artery pressure ≥ 15 mm Hg after 6 months of age. Systemic and pulmonary venous anomalies coupled with single-ventricle anatomy were not significant risk factors for determining a poor outcome of the Fontan procedure.Conclusions. This study suggests that the outcome of the Fontan procedure in patients with heterotaxy syndrome may be improved by early protection of the pulmonary vascular bed, despite the existence of other cardiac anomalies.

The significance of tricuspid regurgitation in hypoplastic left-heart syndrome

Palliation of hypoplastic left-heart syndrome involves use of the morphologic right ventricle as the systemic ventricle and the tricuspid valve (in cases of mitral atresia/stenosis) or the common atrioventricular valve (in cases of malaligned atrioventricular canal) as the systemic atrioventricular valve. To determine the relationship between tricuspid or common atrioventricular valve function and the ultimate outcome of palliative surgery, 100 patients with hypoplastic left-heart syndrome were evaluated preoperatively by Doppler echocardiography to determine the degree of tricuspid regurgitation. These patients were then followed serially to assess changes with time in the functional status of the tricuspid or common atrioventricular valve and to determine the correlation of tricuspid or common atrioventricular valve regurgitation with survival. We discovered that tricuspid or common atrioventricular valve regurgitation is common in hypoplastic left-heart syndrome. Thirty-seven percent of the patients had mild, 13% had moderate, and 3% had severe tricuspid or common atrioventricular valve regurgitation on their preoperative Doppler echocardiograms. Throughout the first 2 postoperative years most patients had no significant change in the degree of tricuspid or common atrioventricular valve regurgitation when findings were compared to those of the preoperative echocardiogram. Patients with moderate or severe tricuspid or common atrioventricular valve regurgitation preoperatively had a significant reduction in their survival when contrasted with patients with no or mild atrioventricular valve regurgitation. We therefore conclude that tricuspid or common atrioventricular valve competence is a significant factor in long-term survival after palliative surgery for hypoplastic left-heart syndrome. This function, however, appears to be unaffected by palliation and remains relatively constant over the first 2 postoperative years.