Biventricular Repair Of Pulmonary Atresia Research Articles

Sacubitril/Valsartan in Adult Congenital Heart Disease Patients With Chronic Heart Failure − A Single Centre Case Series and Call for an International Registry

An improvement in life expectancy in patients suffering from adult congenital heart disease (ACHD) has corresponded with a rise in heart failure incidence within this group. An area that has not been addressed in ACHD heart failure guidelines has been the use of combined inhibition of angiotensin receptor-neprolysin pathways. This case series sought to demonstrate tolerability and 6-month outcome measures of sacubitril/valsartan use in ACHD patients with a severely impaired systemic ventricle. A prospective cohort analysis of ACHD patients between December 2016 and September 2017 with severe systemic ventricular systolic dysfunction, New York Heart Association (NYHA) class II-III symptoms and eligible for commencement on a sacubitril/valsartan regime was undertaken. Five (5) consecutive patients were included in this cohort review, 80% male, mean age 41.8 (±19) years and mean systemic ventricular ejection fraction 27% (±3.6%). Two (2) patients with pre-existing D-TGA and atrial baffle repair, one patient with Tetralogy of Fallot repair and pulmonary valve replacement (PVR), one patient with left atrial isomerism and partial atrioventricular (AV) canal defect repair and mitral valve replacement (MVR) and the last patient had biventricular repair for pulmonary atresia with MVR and PVR. Forty per cent (40%) of patients had a systemic right ventricle. All patients had NYHA functional class of ≥II, were on optimal tolerated doses ACE-I or ARB prior to sacubitril/valsartan combination. Six (6) months post commencement of sacubitril/valsartan patients experienced a mean improvement of one functional class. Our experience suggests that sacubitril/valsartan therapy is well tolerated in ACHD heart failure patients and is associated with improvement in functional class.

Fifty-Five Years Follow-Up of 111 Adult Survivors After Biventricular Repair of PAIVS and PS.

There is paucity of long-term data on adult survivors after biventricular repair of pulmonary atresia with intact ventricular septum (PAIVS) and pulmonary stenosis (PS). This study aimed to determine the cardiac and non-cardiac outcomes of adult survivors after biventricular repair of PAIVS and PS. The cardiac, neurodevelopmental and liver problems of 111 adults, 40 with PAIVS and 71 with PS, were reviewed. The median follow-up duration of our patients was 26.5years (range 14.8-55years). The freedom from reintervention at 30years was 17.4% and 73.3% for PAIVS and PS patients (p < 0.001), respectively. Compared with PS patients, PAIVS patients had significantly greater prevalence of right atrial and right ventricular (RV) dilatation, and moderate to severe tricuspid and pulmonary regurgitation (all p < 0.05), and cardiac arrhythmias (22.5% vs. 8.5%, p = 0.047). The freedom from development of cardiac arrhythmias at 30years of 68.4% and 91.6%, respectively, in PAIVS and PS patients (p = 0.03). Cox proportional hazards model identified PAIVS as an independent risk factor for reintervention (HR 4.0, 95% CI 2.1-7.6, p < 0.001) and development of arrhythmias (HR 4.1, 95% CI 1.1-14.4, p = 0.03). Neurodevelopmental problems were found in 17.5% of PAIVS patients and 7.0% of PS patients (p = 0.11). Liver problems occurred in 2 (5%) PAIVS patients, both of whom required conversion to 1.5 ventricular repair. In conclusion, long-term problems, including the need for reinterventions, cardiac arrhythmias, RV dilation, pulmonary regurgitation, and neurodevelopmental and liver issues are more prevalent in adult PAIVS than PS survivors.

Quantification of Pulmonary Regurgitation by Vector Flow Mapping in Congenital Heart Patients after Repair of Right Ventricular Outflow Obstruction: A Preliminary Study

Vector flow mapping (VFM) enables direct visualization of flow pattern and estimation of flow volume. The aim of this study was to determine its accuracy in the quantification of pulmonary regurgitation (PR) in congenital heart patients after repair of right ventricular (RV) outflow obstruction. This study comprised two parts: (1) validation of VFM in the quantification of PR in patients with repaired tetralogy of Fallot by cardiac magnetic resonance and (2) clinical application of VFM to determine PR in patients after biventricular repair of pulmonary atresia and stenosis with intact ventricular septum. PR was quantified by calculation of VFM-derived pulmonary regurgitant ratio (PRVFM), defined as ratio of backward to forward flow volume. Coefficients of variations for intra- and interobserver variability in the measurements of PRVFM were 7.0% and 10.4%, respectively. Fourteen patients with repaired tetralogy of Fallot aged 31.3±7.3years were studied. Their PRVFM correlated strongly with cardiac magnetic resonance-derived PR fraction (r=0.95, P<.001) and RV end-diastolic volume (r=0.84, P<.001). In the second part, 14 patients with pulmonary atresia with intact ventricular septum aged 25.6±6.0years, 14 patients with pulmonary stenosis aged 24.2±7.0years, and 14 healthy control subjects were studied. PRVFM was found to increase across groups of subjects with absent (4.6±3.3%), mild (11.1±7.1%), moderate (29.6±7.8%), and severe (50.1±8.2%) PR as defined semiquantitatively by color flow mapping. Furthermore, PRVFM correlated strongly with the ratio of PR color jet to width of RV outflow (r=0.92, P<.001). VFM is a reproducible technique for accurate quantification of PR in congenital heart patients after repair of RV outflow obstruction.

純型肺動脈閉鎖術後遠隔期における心臓MRI 検査を用いた両心室収縮機能評価

純型肺動脈閉鎖術後遠隔期における心臓MRI 検査を用いた両心室収縮機能評価

Pulmonary atresia with intact ventricular septum: how good is a biventricular repair?

In this issue of EJCTS, Romeih et al. [1] present some interesting and provocative data regarding long-term outcome for biventricular repair of pulmonary atresia with intact ventricular septum (PAIVS). Debate relative to timing, technique and surgical indications has been going on for decades, and major centres still employ diverse management strategies for this relatively uncommon malformation [2]. The current cross-sectional study is very well conceived and designed, employing state-of-the-art imaging tools to assess exercise capacity and pharmacological stress response in a small group of (asymptomatic) long-term survivors of biventricular repair of PAIVS. Of note, all patients had a tricuspid Z-value >− 2.5 at birth, with 2 or more right ventricular (RV) segments present. This set of qualifications suggests that the study group represents the more favourable end of the spectrum of PAIVS as understood in the era of operation. Whatever the actual right ventricular outflow tract strategy employed, all patients had important pulmonary insufficiency at late followup, despite the universal presence of age-related RV diastolic dysfunction. Reduction in exercise capacity and limitation of response to pharmacological stress were also age-related. From clinical experience, the carefully documented current findings are not unexpected, and, of course, this cohort represents only children qualifying for an early biventricular repair. There is a growing body of data, some cited by the authors, suggesting that Fontan patients (with varying anatomy, and without arrhythmias) can achieve or surpass the exercise capacity of patients undergoing biventricular repair [3]. Of course, this is only a part of the Fontan story, but an important observation nonetheless, and the one which invites comparative studies using the same techniques. Effects of age at initial repair (as opposed to followup interval) and the risk-benefit analysis of valved repair strategies would also be of interest. The authors must be commended on this excellent study which does address some important questions relating to late follow-up for biventricular repair of PAIVS.

Finding the Right Balance Between Univentricular and Biventricular Repair for Pulmonary Atresia with Intact Ventricular Septum

Finding the Right Balance Between Univentricular and Biventricular Repair for Pulmonary Atresia with Intact Ventricular Septum

Restrictive Right Ventricular Physiology and Right Ventricular Fibrosis as Assessed by Cardiac Magnetic Resonance and Exercise Capacity After Biventricular Repair of Pulmonary Atresia and Intact Ventricular Septum

The hypertrophic myocardium, myocardial fiber disarray, and endocardial fibroelastosis in pulmonary atresia and intact ventricular septum (PAIVS) may provide anatomic substrates for restrictive filling of the right ventricle. Restrictive right ventricle (RV) physiology is related to RV fibrosis and exercise capacity in patients after biventricular repair of PAIVS. A total of 27 patients, age 16.5 +/- 5.6 years, were recruited after biventricular repair of PAIVS. Restrictive RV physiology was defined by the presence of antegrade diastolic pulmonary flow and RV fibrosis assessed by late gadolinium enhancement (LGE) cardiac magnetic resonance. Their RV function was compared with that of 27 healthy controls and related to RV LGE score and exercise capacity. Compared with controls, PAIVS patients had lower tricuspid annular systolic and early diastolic velocities, RV global longitudinal systolic strain, systolic strain rate, and early and late diastolic strain rates (all P < 0.05). A total of 22 (81%, 95% confidence interval: 62%-94%) PAIVS patients demonstrated restrictive RV physiology. Compared to those without restrictive RV physiology (n = 5), these 22 patients had lower RV global systolic strain, lower RV systolic and early diastolic strain rates, higher RV LGE score, and a greater percent of predicted maximum oxygen consumption (all P < 0.05). Restrictive RV physiology reflects RV diastolic dysfunction and is associated with more severe RV fibrosis but better exercise capacity in patients after biventricular repair of PAIVS.

Success and limitations of right ventricular sinus myectomy for pulmonary atresia with intact ventricular septum

Right ventricular sinus myectomy has been proposed for pulmonary atresia with intact ventricular septum for morphology falling within the uncertain area for eventual biventricular repair. Our objective was to evaluate right ventricular sinus myectomy by characterizing the morphologic spectrum of these patients, determining whether biventricular repair was achieved, ascertaining growth of right-sided structures, and assessing survival. We evaluated medical records, all imaging studies, and follow-up data (complete in all but 1 patient) from 43 patients with pulmonary atresia with intact ventricular septum treated from October 1993 to July 2005, 16 of whom underwent right ventricular sinus myectomy. Serial echocardiographic measurements of right-sided cardiac structures were converted to Z values to estimate their growth relative to somatic growth. Patients undergoing right ventricular sinus myectomy had mild-to-moderate right ventricular size diminution (grade -1.2 +/- 3.2) and a tricuspid valve Z value of -4.9 +/- 1.9. Thirteen (87%) of the 16 patients achieved biventricular repair. After right ventricular sinus myectomy, mean right ventricular cavity size grade increased to 1.4 +/- 0.66, but the tricuspid valve Z value did not change appreciably over time. Five-year survival after sinus myectomy was 85%; late deaths were in patients with the smallest tricuspid valves at presentation (Z value < -7). Right ventricular sinus myectomy in the uncertain area for biventricular repair of pulmonary atresia with intact ventricular septum leads to immediate increase in right ventricular volume. It, in combination with establishing right ventricle-pulmonary trunk continuity, allowed early biventricular repair in 87% of patients. However, tricuspid valve growth in relation to somatic growth was minimal. Thus, small tricuspid valve size might limit the long-term success of biventricular repair achieved by means of right ventricular sinus myectomy.

Mounting Evidence That Fibrosis Generates a Major Mechanism for Atrial Fibrillation

See related article, pages 839–847 It is widely known that atrial fibrillation (AF) is the most common sustained cardiac arrhythmia, with the problem magnified by the clinical sequelae; eg, thromboembolic events. It is pertinent that the incidence of AF is expected to increase in the future,1 especially in aging patients. Thus, finding preventive measures for this arrhythmia has become an increasingly important goal. In addition to considering clinical risk factors, focusing on prevention necessitates knowledge of the associated pathophysiology of AF,2 which involves the initiation of AF and the wavefront dynamics that sustain it. In recent years considerable new information has appeared concerning AF mechanisms that occur in different regions of the atria in different cardiac states.3 This point is highlighted by the report of Tanaka et al4 in this issue of Circulation Research . These authors used high-resolution electrophysiological and microstructural techniques, along with computer model simulations, to study wavefront dynamics during acetylcholine (ACh)-induced AF in heart failure sheep hearts. The heart failure hearts had developed prominent fibrotic patches in the posterior left atrium near the pulmonary veins, whereas in the control (normal) hearts patches of fibrosis were smaller, diffusely distributed, and more centrally located with respect to the 4 pulmonary vein ostia. In the heart failure hearts, during AF variable wavefront breakthroughs to the endocardium occurred in the area of fibrotic patches adjacent to the pulmonary veins. The authors concluded that scroll waves within the posterior left atrial wall produced a microreentry source for the endocardial breakthroughs in the region of the larger collagen patches, thus providing the underlying mechanism of AF.4 A myriad of reports provide varied information about substrates and mechanisms of AF as a background for the study of Tanaka et al …

Fertility, Pregnancy, and Delivery After Biventricular Repair for Pulmonary Atresia With an Intact Ventricular Septum

The objective of the present study to investigate fertility, pregnancy, and delivery in women with biventricular repair for pulmonary atresia with an intact ventricular septum (PAIVS). Using a nationwide registry (CONCOR), 37 patients with pulmonary atresia were identified, 6 of whom (aged 21 to 34 years) had biventricular repair for PAIVS. Three PAIVS patients had a total of 5 pregnancies, including 1 abortion and 1 ectopic pregnancy. Besides minor noncardiac complications, the 3 live-birth pregnancies were successful. None of the women reported infertility or irregularities (hypermenorrhea, dysmenorrhea, polymenorrhoea, oligomenorrhea, or amenorrhea) of their natural menstrual cycle. In conclusion, successful pregnancy in women with biventricular repair for PAIVS is possible, and only minor complications were observed. Infertility and menstrual cycle disorders do not appear to be more prevalent than usual.

Pregnancy After Biventricular Repair for Pulmonary Atresia With Ventricular Septal Defect

Information on pregnancy and delivery in women with biventricular repair for isolated noncomplex pulmonary atresia with a ventricular septal defect (PAVSD) is limited. Using a nationwide congenital heart disease registry (CONgenital CORvitia [CONCOR]), 9 women with biventricular repair for PAVSD (aged 21 to 38 years) were identified. Ten pregnancies were observed in 5 different women with PAVSD, including 3 spontaneous miscarriages. Clinically significant (non)cardiac complications were documented in 3 of 7 completed pregnancies. These complications were: (1) atrioventricular reentry tachycardia with symptomatic right-sided heart failure; (2) eclampsia with hemolysis, elevated liver enzymes, and low platelets syndrome further complicated by abruptio placentae leading to premature delivery of a small-for-gestational-age child; and (3) premature delivery due to cervical insufficiency with antepartum demise of an immature child. Furthermore, none of the women reported infertility. Moreover, none of the women reported irregularities of their natural menstrual cycle (age at menarche 13 years; cycle duration 28 days), with the exception of delayed menarche (>16 years) in 2 patients. In conclusion, successful pregnancy in patients with biventricular repair of PAVSD is possible, although often complicated by serious clinically significant events. Infertility and menstrual cycle disorders do not appear to be more prevalent, except for a high incidence of primary amenorrhea.

Risk factors for early death and reoperation following biventricular repair of pulmonary atresia with intact ventricular septum

Since a functional right ventricle is desirable when repairing pulmonary atresia with intact ventricular septum, we sought to determine the factors that portend a successful biventricular repair in these children. A review of operative records at UCLA between 1982 and 2001 revealed 56 patients diagnosed with pulmonary atresia with intact ventricular septum that underwent either a partial (n=26) or complete biventricular repair. Kaplan-Meier survival curves with log rank statistics were used to evaluate the influence of demographic, technical, and anatomic factors on survival and need for reoperation. Five-year actuarial survival following biventricular repair was 91.5%. Non-Caucasian race (p=0.011) and omission of palliative right ventricular outflow tract obstruction (RVOTO) relief (p=0.042) were risk factors for early death. All patients with adequate follow-up required reoperation with median duration of 6.9 years. The most common cause of early reoperation (<1 year) was wound infection (35.3%) while pulmonary valve replacement (58.8%) constituted the majority of late reoperations. Risk factors for wound infection included female sex (p=0.011) and use of a synthetic transannular patch (p=0.085). The most significant risk factor for early pulmonary valve replacement was the use of a monocusp pericardial valve in the pulmonary annular position (p=0.003). Excellent survival follows biventricular repair of pulmonary atresia with intact ventricular septum if RVOTO relief is performed in conjunction with initial palliation. Although most patients eventually require reoperation, avoidance of synthetic transannular patches and monocusp pericardial pulmonary valves may reduce the incidence of wound infection and delay need for pulmonary valve replacement.

Assessment of right and left ventricular function by tissue Doppler echocardiography in patients after biventricular repair of pulmonary atresia with intact ventricular septum

Background Survival of patients with PAIVS has improved significantly with adoption of a selective management approach based on RV and coronary arterial anatomy. We sought to determine the right (RV) and left ventricular (LV) function of patients with pulmonary atresia and intact ventricular septum (PAIVS) long-term after biventricular repair. Methods The regional ventricular function of 22 patients (11 males) aged 12.8 ± 5.6 years with PAIVS after biventricular repair was assessed by tissue Doppler echocardiography (TDE). The results were compared to those of 22 age-matched controls. Results When compared with controls, patients had significantly reduced myocardial tissue velocities, systolic and diastolic strain rate, and peak systolic strain in the basal, mid-, and apical segments of the RV free wall, interventricular septum and LV lateral wall (all with p < 0.01). Heterogeneity of systolic and diastolic strain rate was observed in the RV free wall and interventricular septum in patients but not in controls. Conclusion Impairment of RV and LV regional longitudinal myocardial function occurs in patients with PAIVS late after biventricular repair.

Staged biventricular repair of pulmonary atresia or stenosis with intact ventricular septum

Background. Since 1991 we have performed a multistage palliative approach to biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum in infants with a detectable right ventricular infundibulum. Methods. A total of 25 patients (19 pulmonary atresia and 6 critical pulmonary stenosis) underwent initial palliation consisting of a transarterial pulmonary valvotomy and a polytetrafluoroethylene shunt between the left subclavian artery and pulmonary trunk. Among the 23 survivors, 15 underwent balloon valvotomy. Six of these patients later required additional palliative surgery that consisted of repeat pulmonary valvotomy, adjustment of an atrial communication, and resection of the hypertrophied muscles in the right ventricle. Results. Of the 25 patients, 23 (92%) survived. In all, 20 patients underwent definitive operations: 18 (90%) biventricular repair (12 pulmonary atresia, and 6 critical pulmonary stenosis), one bidirectional Glenn, and one Fontan procedure. The actuarial probability of achieving a biventricular repair at 36 months of age was 69%. In 18 patients right ventricular end–diastolic volume significantly increased but tricuspid valve diameter did not change. Conclusions. The multistage palliation procedure to promote right ventricular growth makes a definitive biventricular repair of pulmonary atresia or critical pulmonary stenosis with intact ventricular septum possible in the majority of infants with a patent infundibulum.

Long-term outcome for right heart function after biventricular repair of pulmonary atresia and intact ventricular septum

The right heart function of the patients with pulmonary atresia and intact ventricular septum was assessed periodically during the process of staged biventricular repair, and the implications for its long-term outcome were analyzed. During the period from 1971 to 1990, 21 neonates or infants with pulmonary atresia and intact ventricular septum had undergone initial palliative surgery. There were seven early postoperative deaths and one late death. Of the 13 survivors, 10 patients underwent subsequent biventricular repair and form the basis of this study. Their clinical records of roentgenography, electrocardiography, and catheterization studies at each staged period were reviewed retrospectively. Arrhythmia occurred late in 2 patients, one of whom died by arrhythmia at 11 years after definitive surgical repair. Therefore the actuarial survival rate was 85.7% at 14 years. The catheterization study after the definitive biventricular repair revealed a significant fall in the right heart pressure (p = 0.0005) and significant improvement in the right ventricular ejection fraction (p = 0.0004). In angiocardiography, dilatation of the right atrium was noted in all patients and was more marked in those who developed arrhythmia in conjunction with rapid growth of the right ventricle. Moreover, the serial repeated electrocardiography disclosed progressive and significant prolongation of both PQ interval (p = 0.003) and QRS duration (p = 0.021). Although biventricular repair for pulmonary atresia and intact ventricular septum proved to attain a satisfactory long-term result, it failed to resolve right heart dysfunction. Postoperative arrhythmia was prone to precipitate progressive dilatation of the right atrium.

Thirty-sixth Annual Meeting January 31–February 2, 2000 Greater Fort Lauderdale/Broward County Convention Center, Fort Lauderdale, Florida

Thirty-sixth Annual Meeting January 31–February 2, 2000 Greater Fort Lauderdale/Broward County Convention Center, Fort Lauderdale, Florida