Systemic Atrioventricular Valve Regurgitation Research Articles

Combined Norwood and Modified Left-Sided Starnes Procedures for Congenitally Corrected Transposition of the Great Arteries.

The combination of congenitally corrected transposition of the great arteries (ccTGA), Ebstein anomaly of the systemic atrioventricular valve, and critical systemic ventricular outflow tract obstruction is extremely rare. Management of a neonate with these lesions in cardiogenic shock is quite challenging. Tricuspid valve exclusion has been increasingly and successfully used to manage neonatal Ebstein anomaly patients. We extended this idea of tricuspid valve exclusion (modified Starnes) combined with a Norwood Stage 1 operation with a modified systemic to pulmonary artery shunt to successfully manage a neonate with ccTGA, severe systemic atrioventricular valve regurgitation, and critical ventricular outflow tract obstruction.

Longitudinal analysis of systemic ventricular function and atrioventricular valve function after the Norwood procedure.

To evaluate longitudinal systemic ventricular function and atrioventricular valve regurgitation in patients after the neonatal Norwood procedure. Serial postoperative echocardiographic images before Fontan completion were assessed in neonates who underwent the Norwood procedure between 2001 and 2020. Ventricular function and atrioventricular valve regurgitation were compared between patients with modified Blalock-Taussig shunt and right ventricle to pulmonary artery conduit. A total of 335 patients were identified including 273 hypoplastic left heart syndrome and 62 of its variants. Median age at Norwood was 8 (7-12) days. Modified Blalock-Taussig shunt was performed in 171 patients and the right ventricle to pulmonary artery conduit in 164 patients. Longitudinal ventricular function and atrioventricular valve regurgitation were evaluated using a total of 4352 echocardiograms. After the Norwood procedure, ventricular function was initially worse (1-30 days) but thereafter better (30 days to stage II) in the right ventricle to pulmonary artery conduit group (P < 0.001). After stage II, the ventricular function was inferior in the right ventricle to the pulmonary artery conduit group (P < 0.001). Atrioventricular valve regurgitation between the Norwood procedure and stage II was more frequent in the modified Blalock-Taussig shunt group (P < 0.001). After stage II, there was no significant difference in atrioventricular valve regurgitation between the groups (P = 0.171). The effect of shunt type on haemodynamics after the Norwood procedure seems to vary according to the stage of palliation. After the Norwood, the modified Blalock-Taussig shunt is associated with poorer ventricular function and worse atrioventricular valve regurgitation compared to right ventricle to pulmonary artery conduit. Whereas, after stage II, modified Blalock-Taussig shunt is associated with better ventricular function and comparable atrioventricular valve regurgitation, compared to the right ventricle to pulmonary artery conduit.

Systemic Atrioventricular Valve Surgery in Patients With Congenitally Corrected Transposition of the Great Vessels.

Limited data exist regarding the long-term outcomes of systemic atrioventricular valve (SAVV) intervention (morphologic tricuspid valve) in congenitally corrected transposition (ccTGA). The goal of this study was to evaluate the mid- and long-term outcomes of SAVV surgery in ccTGA. We performed a retrospective review of 108 ccTGA patients undergoing SAVV surgery from 1979 to 2022. The primary outcome was a composite endpoint of mortality, cardiac transplantation, or ventricular assist device implantation. The secondary outcome was long-term systemic right ventricular ejection fraction (SVEF). Cox proportional hazard and linear regression models were used to analyze survival and late SVEF data. The median age at surgery was 39.5 years (Q1-Q3: 28.8-51.0 years), and the median preoperative SVEF was 39% (Q1-Q3: 33.2%-45.0%). Intrinsic valve abnormality was the most common mechanism of SAVV regurgitation (76.9%). There was 1 early postoperative mortality (0.9%). Postoperative complete heart block occurred in 20 patients (18.5%). The actuarial 5-, 10-, and 20-year freedom from death or transplantation was 92.4%, 79.1%, and 62.9%. The 10- and 20-year freedom from valve reoperation was 100% and 93% for mechanical prosthesis compared with 56.6% and 15.7% for bioprosthesis (P< 0.0001). Predictors of postoperative mortality were age at operation (P=0.01) and preoperative SVEF (P=0.04). Preoperative SVEF (P< 0.001), complex ccTGA (P=0.02), severe SAVV regurgitation (P=0.04), and preoperative creatinine (P=0.003) were predictors of late postoperative SVEF. SAVV surgery remains a valuable option for the treatment of patients with ccTGA, with low early mortality and satisfactory long-term outcomes, particularly in those with SVEF≥40%. Timely referral and accurate patient selection are the keys to better long-term outcomes.

Considerations of valvular heart disease in children with ventricular assist devices.

Ventricular assist devices have become a valuable tool in the treatment of heart failure in children. The use of ventricular assist devices has decreased mortality in children with end-stage heart failure awaiting transplant. It is not uncommon for children with end-stage heart failure associated with cardiomyopathy or congenital heart disease to have significant systemic semilunar and atrioventricular valve regurgitation, which can impact the efficiency and efficacy of hemodynamic support provided by a ventricular assist device. Therefore, implanting clinicians should carefully assess for valve abnormalities that may need repair and impact device selection and cannulation strategy to effectively support this diverse population. The purpose of this review is to provide an overview of this important and relevant topic and to discuss strategies for managing these patients.

Longitudinal analysis of systemic ventricular function and atrioventricular valve function after the Fontan procedure.

This study aimed to determine the longitudinal change of systemic ventricular function and atrioventricular valve (AVV) regurgitation after total cavopulmonary connection (TCPC). In 620 patients who underwent TCPC between 1994 and 2021, 4219 longitudinal echocardiographic examinations of systemic ventricular function and AVV regurgitation were evaluated retrospectively. The most frequent primary diagnosis was hypoplastic left heart syndrome in 172, followed by single ventricle in 131, tricuspid atresia in 95 and double inlet left ventricle (LV) in 91 patients. Dominant right ventricle (RV) was observed in 329 (53%) and dominant LV in 291 (47%). The median age at TCPC was 2.3 (1.8-3.4) years. Transplant-free survival at 5, 10 and 15 years after TCPC was 96.3%, 94.7% and 93.6%, respectively, in patients with dominant RV and 97.3%, 94.6% and 94.6%, respectively, in those with dominant LV (P = 0.987). Longitudinal analysis of systemic ventricular function was similar in both groups during the first 10 years postoperatively. Thereafter, systemic ventricular function worsened significantly in patients with dominant RV, compared with those with dominant LV (15 years: P = 0.007, 20 years: P = 0.03). AVV regurgitation was more frequent after TCPC in patients with dominant RV compared with those with dominant LV (P < 0.001 at 3 months, 3 years, 5 years, 10 years and 15 years, P = 0.023 at 20 years). There was a significant correlation between postoperative systemic ventricular dysfunction and AVV regurgitation (P < 0.001). There were no transplant-free survival difference and no difference in ventricular function between dominant RV and dominant LV for the first 10 years after TCPC. Thereafter, ventricular function in dominant RV was inferior to that in dominant LV. The degree of AVV regurgitation was significantly higher in dominant RV, compared with dominant LV, and it was positively associated with ventricular dysfunction, especially in dominant RV.

Contemporary risk factors for a longer hospital stay following bidirectional cavopulmonary anastomosis.

Despite high survival after bidirectional cavopulmonary anastomosis, a considerable number of patients suffer significant post-operative morbidities related to prolonged length of stay. A single-center retrospective cohort study of all consecutive patients undergoing a first-time bidirectional cavopulmonary anastomosis from 2006 to 2019. Prolonged length of stay was defined as hospital stay greater than the 75th percentile for our cohort. Of 195 patients who met inclusion criteria, the median post-operative length of stay was 8 days (interquartile range, 4-15 days). Prolonged length of stay was defined as greater than 15 days. In multivariate analysis, greater than mild systemic atrioventricular valve regurgitation (odds ratio 3.7, 95% CI 1.05-13.068, p = 0.04), longer length of stay after the initial palliative procedure (odds ratio 1.028, 95% CI 1.004-1.05, p = 0.02), and pre-operative higher superior vena cava oxygen saturation (odds ratio 0.922, 95% CI 0.85-0.99, p = 0.04) maintained statistical significance as independent risk and protective factors for prolonged length of stay. A one-level increase in the severity of pre-operative systemic atrioventricular valve regurgitation was associated with a multiplicative change in the odds ratio of prolonged length of stay of 5.45 (p = 0.005) independent of the severity of systemic ventricular dysfunction. Pre-operative characteristics with greater than mild systemic atrioventricular valve regurgitation, longer length of stay after the initial palliative procedure, and lower superior vena cava oxygen saturation were associated with prolonged length of stay after a first-time bidirectional cavopulmonary anastomosis.

Intermediate-term outcomes of deferred Norwood strategy.

The aim of this study was to assess outcomes of the deferred Norwood strategy, i.e. planned Norwood following routine bilateral pulmonary artery banding and ductal stenting or continuous prostaglandin E1 administration. Forty-five patients with hypoplastic left heart syndrome and its variants treated with the deferred Norwood strategy between 2012 and 2021 were enrolled. Mid-term outcomes were retrospectively reviewed. The median follow-up period after Norwood in hospital survivors was 4.6 years (interquartile range: 1.9, 6.8). Fourteen patients (31.1%) had no risk factors. The median age and weight at Norwood were 1.8 months (0.9, 3.5) and 3.1 kg (2.7, 3.6). Transplant-free survival at 6 years was 84.5%. Birth weight ≤2.5 kg and systemic atrioventricular valve (SAVV) regurgitation ≥ moderate after birth were not risk factors for mortality; body weight at Norwood ≤2.5 kg, however, was a risk factor [hazard ratio (HR), 11.3; 95% confidence interval (CI), 1.2-11; P = 0.036]. Twenty-two (48.9%) underwent Fontan with no mortalities, and 7 (15.5%) are awaiting Fontan. Freedom from SAVV surgery at 5 years was 53.1%. SAVV regurgitation ≥ moderate after birth was a risk factor for SAVV surgery (HR, 16; 95% CI, 3.6-71; P < 0.001); however, ductal stenting had a protective effect against SAVV surgery (HR, 0.09; 95% CI, 0.01-0.68; P = 0.019). Freedom from both surgical and catheter-based pulmonary artery intervention at 3 years was 27.1. Although deferred Norwood provided acceptable intermediate-term survival, the Fontan completion rate was unsatisfactory. SAVV surgery and pulmonary artery intervention were frequently required.

Biomarkers for heart failure and prognostic prediction in patients with Fontan circulation.

A wide variety of pathologies are involved in heart failure in patients with congenital heart disease (CHD). Specific causes of heart failure after the Fontan procedure include not only single-ventricle circulation, but also the function of the right ventricle as the systemic ventricle, atrioventricular or semilunar valve stenosis or regurgitation, pulmonary hypertension, and left ventricular dysfunction secondary to right ventricular enlargement or dysfunction. As heart failure can occur post-Fontan, for a variety of reasons, clarification of the pathophysiology is the first step in management and treatment. At the same time, it is important to understand each patient's current condition and treatment plan to make an accurate prognosis. Because of the wide variety of pathophysiologies in post-Fontan CHD patients, however, no single biomarker is useful in all situations. Relevant biomarkers must be selected according to each patient's disease state, and combinations of multiple biomarkers should also be considered. In this review, the author describes the clinical importance of various biomarkers for patients who have undergone a Fontan procedure.

Late outcome, therapy and systemic ventricular function in patients with a systemic right ventricle: data of the German National Register for Congenital Heart Defects.

Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure. A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included. Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation. A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.

Management of Congenitally Corrected Transposition of the Great Arteries With Intact Ventricular Septum: Anatomic Repair or Palliative Treatment?

[Figure: see text].

Adverse functional remodelling of the subpulmonary left ventricle in patients with a systemic right ventricle is associated with clinical outcome.

Early recognition of adverse remodelling is important since outcome is unfavorable once patients with a systemic right ventricle (sRV) become symptomatic. We aimed assessing prognostic markers linked to short-term clinical evolution in this population. Thirty-three patients (76% male) with sRV (atrial switch repair for D-transposition of the great arteries and congenitally corrected transposition of the great arteries) underwent detailed phenotyping including exercise cardiac magnetic resonance and were followed over mean follow-up time of 3 years. Mean age was 40 ± 8 (range 26-57) years at latest follow-up. Adverse outcome was a composite of heart failure (HF) and tachyarrhythmia. Descriptive statistics and univariate cox regression analyses were performed. When compared with baseline: (i) most patients remained in New York Heart Association functional class I (76%), (ii) the degree of severity of the systemic atrioventricular valve regurgitation rose, and (iii) more electrical instability was documented at latest follow-up. Six (18%) of a total of 9 events were counted as first cardiovascular events (9% HF and 9% arrhythmia). NT-proBNP, oxygen pulse, left ventricle end-diastolic volume index (LVEDVi), and stroke volume index (SVi) of the subpulmonary left ventricle (LV) both in rest and at peak exercise were significantly associated with the first cardiovascular event. NT-proBNP was by far the best prognostic marker for clinical outcome. Adverse remodelling with increase of LVEDVi and SVi of the subpulmonary LV at rest and during exercise were associated with worse clinical outcome. We theorize that remodelling of the subpulmonary ventricle might be an early sign of a failing sRV circulation.

Long-Term Outcomes After Fenestration Closure in High-Risk Fontan Candidates.

The study aimed to assess the long-term outcomes after fenestration closure in patients at risk for Fontan failure. Of 119 patients who underwent Fontan operation between 1995 and 2004, fenestration was not created in 89 patients (NF group) and created in 30 patients with hypoplastic left heart syndrome, heterotaxy syndrome, high pulmonary arterial pressure, high systemic ventricular end-diastolic pressure, low ventricular ejection fraction, or atrioventricular valve regurgitation. All fenestrations were closed spontaneously or by catheter/surgical interventions, excepting two patients, and therefore, they were excluded. In fenestration group, patients with pre-Fontan mean pulmonary arterial pressure ≥ 15mmHg or systemic atrioventricular valve regurgitation ≥ moderate were classified as high-risk Fontan candidates (F-HR group, n = 16), and the remaining patients were as standard-risk (F-SR group, n = 12). Protein-losing enteropathy-free survival rates did not differ among the three groups (p = 0.72). Serial follow-up catheter examinations after Fontan operation were completed in 69 patients in NF group and 11 patients in both F-SR and F-HR groups. Cardiac index and pulmonary vascular resistance significantly and similarly decreased over time in all groups, though the F-HR group showed lowest arterial oxygen saturation, lowest cardiac index, and highest pulmonary vascular resistance. The F-HR group also showed much veno-venous collaterals (p = 0.049), low peak oxygen consumption (p = 0.019), and low anaerobic threshold (p = 0.023) as compared to those in the F-SR group. In F-HR group, cyanosis remained after fenestration closure due to transformation from fenestration to veno-venous collaterals, which resulted in elevation of pulmonary vascular resistance, low cardiac index, and deterioration of exercise tolerance.

Prevalence of pulmonary hypertension in adults after atrial switch and role of ventricular filling pressures

ObjectiveTo assess the prevalence of elevated systemic right ventricular (sRV) end-diastolic pressure and pulmonary arterial hypertension in adults with transposition of the great arteries (TGA) who have undergone atrial switch...

The Effect of Hypoxic Gas Therapy Using Nitrogen Before Bilateral Pulmonary Artery Banding on Hemodynamics and Pulmonary Artery Development

This study aimed to identify its influence on pulmonary development and hemodynamics before bidirectional Glenn (BDG) anastomosis. This retrospective study involved 30 consecutive patients with univentricular hearts who underwent bilateral pulmonary artery banding (bil-PAB) and BDG anastomosis between September 2011 and September 2018 at our institution. These patients were classified into the nitrogen (N = 12) and no nitrogen (N = 18) groups according to their use of hypoxic gas therapy. Clinical echocardiographic, operative, and catheter examination data were reviewed. Nakata index was 131 (110-167) in the nitrogen group and 138 (88.4-161.7) in the no nitrogen group (P = 0.8). Pulmonary vascular resistance (PVR) was 1.50 (1.30-2.4) in the nitrogen group and 138 (88.4-161.7) in the no nitrogen group (P = 0.3). There was no statistical difference between the pulmonary development in both groups. In the subgroup of hypoplastic left heart syndrome patients, there was a statistical difference. RVEDV% of normal was 232.5 (215-239) in the nitrogen group and 201 (161-209) in the no nitrogen group (P = 0.03). Pulmonary development was not significantly different when hypoxic gas therapy was performed using nitrogen vs. not using nitrogen. However, RVEDV% of normal might be larger with hypoxic gas therapy using nitrogen. Although there is no obvious systemic atrioventricular valve regurgitation exacerbation with hypoxic gas therapy, it might be useful to prevent a decrease in PVR and may also affect bil-PAB and subclinical increase in systemic atrioventricular valve regurgitation.

Abstract 17117: Prevalence of Systemic AV Valve Regurgitation in Patients With Congenitally Corrected Transposition of the Great Arteries: Anatomic versus Physiologic Repair

Background: Congenitally corrected transposition of the great arteries (ccTGA) is a rare anomaly with highly variable time of diagnosis. Depending on time of diagnosis and associated lesions, multiple therapies exist, directed towards surgical correction. Anatomic repair (AR) involving double switch or atrial switch/Rastelli and physiologic repair (PR) leaving the right ventricle as the systemic ventricle are the two main surgical options. A subset of patients without associated lesions exist who can be followed expectantly over time. Systemic atrioventricular valve (SAVV) function is a major anatomic variable, determining eligibility for double switch and durability of physiologic repair or expectant management (medical group). We therefore sought to assess the prevalence of AV valve regurgitation in the different subgroups and its determinants, hypothesizing that SAVV regurgitation (SAVVR) was reduced in the AR group. Methods: Retrospective record review coupled with prospective cross-sectional follow-up of 240 patients with ccTGA treated at our center was performed. Echocardiographic imaging review was conducted to assess SAVV function and systemic ventricular function among the 3 subgroups. Results: Of 240 patients with ccTGA, 162 met inclusion. Median follow up was 10 years (mean +/- SD 11+/- 9 years). In the AR group majority (85%) had none/mild SAVVR. Prior PA band did not influence the severity of SAVVR following double switch. In the PR group, 22% underwent SAVV repair/replacement, and 75% had none/mild SAVVR; as opposed to only 65% with none/mild SAVVR in the medical group. Only 14% of patients were free of systemic ventricle dysfunction in PR group as opposed to 72% in the AR group. Conclusions: SAVV function is preserved in the AR group. In contrast patients in the PR group had higher prevalence of more than mild SAVVR followed by patients who were managed expectantly.

The Fate of Congenitally Corrected Transposition of the Great Arteries Unoperated Before Adulthood

The outcomes, therapeutic strategies, and risk factors of congenital corrected transposition of great arteries (ccTGA) unoperated before adulthood are unclear. From October 2009 to January 2018, 117 adult ccTGA patients, classified into ccTGA with intact ventricular septum, ventricular septum defect, and pulmonary valve or subpulmonary outflow tract stenosis (PS) groups, were reviewed. Statistical analysis was performed with SPSS 19.0 (IBM, Armonk, NY). At the first visit, no patients suffered operation. The PS group had the least systemic atrioventricular valve regurgitation and the greatest systemic ventricular ejection fraction. All 49 patients underwent surgery. From the first visit to last follow-up, systemic ventricular ejection fraction of unoperated ccTGA decreased significantly. In the intact ventricular septum group, patients receiving systemic atrioventricular valve replacement/valvuloplasty had a significantly increased systemic ventricular ejection fraction and statistically more freedom from death and transplant than unoperated. In the ventricular septum defect group the late systemic ventricular ejection fraction of operated patients was not statistically different from their basic data at first visit. In the PS group patients receiving physiologic repair had significantly decreased systemic ventricular ejection fractions. Severe systemic atrioventricular valve regurgitation, physiologic repair, and systemic ventricular dysfunction (ejection fraction <40%) were risk factors for mortality, transplant, and congestive heart failure. PS protects against systemic atrioventricular valve regurgitation and ventricular dysfunction. Systemic atrioventricular valve replacement/valvuloplasty improved systemic ventricular function for ccTGA with an intact ventricular septum. Physiologic repair was not ideal for ccTGA with PS. Severe systemic atrioventricular valve regurgitation and systemic ventricular dysfunction were associated with suboptimal outcomes.

Patients with Fontan circulation beyond the fourth decade of life

Abstract Background The survival rate after Fontan procedure is improving and many of these patients now survive well into adulthood. Data on the late morbidity and mortality of Fontan patients in their fourth decade of life are lacking. Purpose To evaluate the late outcome of adult patients with Fontan circulation who have survived beyond 35 years. Methods Data were collected retrospectively on consecutive patients with a Fontan circulation ≥35 years between 2005 and 2019. Baseline (at 35th birthday) and follow-up data includes functional class, blood tests, history of arrhythmia, heart failure (HF), thromboembolism, Fontan related liver disease (FLD), protein losing enteropathy (PLE) and plastic bronchitis. Echocardiographic data on systemic atrioventricular valve (SAVV) regurgitation and ventricular function were also analysed. Results 61 patients were included (29, 47.5% female). Tricuspid atresia was the most common underline anatomy (29,47.5%) and the median age at Fontan procedure was 12 [8.0–19.0] years. 34 (56%) patients had an atrio-pulmonary connection Fontan and 15 (24.5%) total cavo-pulmonary connection (86.7% lateral tunnel and 13.3% extra-cardiac conduit), 10 (16%) a Bjork Fontan and 2 (3%) a Kawashima procedure. At baseline 40 (66%) patients were in NYHA class II or III, 7 (11%) had a moderate or severely impaired ventricular function and 12 (20%) had at least moderate SSAV regurgitation. At baseline, 35 (57.4%) patients had experienced at least one arrhythmia, 7 (11.4%) patients had a history of atrial thrombosis or thromboembolic events and 2 (3.3%) of PLE. At a median follow-up of 6.4 [2.9–11.3] years, 11 (18%) patients died, 7 of chronic HF, 1 hepatic carcinoma, 1 sepsis, 1 pulmonary embolism, 1 malignancy (1 cause unknown). During follow-up, 28 (46%) patients had a new episode of atrial arrhythmia, 23 (38%) developed FLD and 2 (3.3%) PLE. 9 (15%) required at least one admission for HF. 2 (3.3%) patients experienced worsening of their ventricular function and 7 (11.4%) of their SAVV regurgitation. On univariable analysis, a baseline diagnosis of PLE (HR 15.23, 95% CI:2.77–83.86, p=0.002), HF (HR 6.18, 95% CI:1.74–21.99, p=0.005), atrial arrhythmia (HR 4.99, 95% CI:1.07–23.21, p=0.04), lower serum albumin (HR 1.14, 95% CI:1.06–1.23, p=0.0006), lower iron levels (HR 1.31, 95% CI:1.02–1.68, p=0.04) and a higher urea (HR 1.18, 95% CI:1.04–1.34, p=0.01) were associated with death or heart transplantation. On bivariable analysis, lower serum albumin was stronger than all other univariable predictors. Conclusions Adult Fontan survivors are a high risk population with significant morbidity and mortality driven primarily by HF and other long-term complications of the Fontan operation. The predictors of outcome in this cohort reflects the chronic low cardiac output state and systemic venous hypertension. Vigilance is required to identify patients who may benefit of haemodynamic optimization and close monitoring for Fontan-related complications. Funding Acknowledgement Type of funding source: None

Long term follow-up after pregnancy in Fontan patients

Abstract Background Patients with single ventricle physiology who have benefited from the Fontan procedure are reaching childbearing age. In this population, complications are common around pregnancy, but the long term effect of pregnancy is unclear. Purpose To evaluate functional and structural changes several years after pregnancy in patients with Fontan circulation. Methods Women with Fontan circulation who had a pregnancy beyond the 20 weeks of gestation followed at our centre between 2005–2019 were included. The following data were collected before pregnancy and at the last follow up (f-up): cardiac anatomy, type of Fontan procedure, resting O2 saturation (O2sat), NYHA class, systemic atrioventricular valve (SAVV) regurgitation, ventricular function, history of arrhythmias, heart failure (HF), thromboembolism, Fontan-related liver disease (FLD), protein-losing enteropathy (PLE), plastic bronchitis and cardiopulmonary test data. Results 12 patients had 18 pregnancies, giving birth to 17 live newborns (1 newborn death at 24 weeks of gestation). 7 (58.3%)patients had a total cavo-pulmonary connection, 3 (25%)an atrio-pulmonary connection and 2 (16.7%)a Bjork type Fontan. Tricuspid atresia was the most common anatomy (6, 50%). Before pregnancy, O2sat was 95.4±1.9%and all patients were in NYHA class I or II. All patients (12, 100%)had a normal or mildly impaired ventricular function and 3 (25%)had a moderate or severe SAAV regurgitation. 6 (50%)patients had 2 pregnancies. Mean age at first pregnancy was 29.9±4.4 years. Cardiac events occurred in 7 (38%)pregnancies, most commonly atrial arrhythmia (4, 57%), 2 (16.6%)patients developed new cyanosis and 1 had a thromboembolic event. No maternal death occurred and 1 patient had severe haemorrhage. After a mean f-up of 84±48 months, there was no significant worsening in O2sat (94.3±3.1 at f-up, p=1) and all patients remained in NYHA class I or II (12, 100%, p=1). At f-up all patients (12, 100%)continued to have a normal or mildly impaired ventricular function (p=1) and there was no significant worsening in the SAAV regurgitation (3 patients with moderate or severe regurgitation, p=1). 3 (25%)patients developed FLD (p=0.3), 3 (25%) a new episode of atrial arrhythmia (p=0.3). There were no significantly differences in peak VO2 (22.5±3.8 pre-pregnancy, 22.8±5.5 at f-up, p=0.4) and VE/VCO2 slope (34.9±11.2 pre-pregnancy, 36.3±11.8 at f-up, p=0.2)before pregnancy and during f-up. Conclusions In this small cohort of Fontan patients who successfully completed at least 1 pregnancy, there was no significant decline in functional or structural cardiac parameters long term after delivery. Funding Acknowledgement Type of funding source: None

Percutaneous mitral valve repair in adults with congenital heart disease: Report of the first case-series.

Systemic atrioventricular valve regurgitation (AVVR) is frequently encountered in adults with congenital heart disease (CHD). Surgical intervention is the mainstay of therapy, but in a specific high-risk subset, percutaneous valve repair might offer a lower-risk alternative. Three patients with complex CHD and severe symptomatic AVVR underwent percutaneous mitral valve repair at a single center. All were deemed to be high-risk for surgery by a multidisciplinary CHD team and provided informed consent for the compassionate use of the MitraClip (Abbott, Santa Clara, CA). Three-dimensional heart models were generated for the procedure, which was performed by an adult CHD cardiologist (who provided imaging support) and an interventional cardiologist with expertise in CHD and percutaneous mitral valve repair. The first case was a 39 year-old-woman with [S,L,D] dextrocardia, double outlet right ventricle, mild tricuspid hypoplasia, and a secundum atrial septal defect, who was palliated at age 35 with a right bidirectional Glenn and later developed severe, symptomatic mitral regurgitation, and underwent placement of one MitraClip XTR device. Two patients with L-loop transposition of the great arteries each successfully underwent placement of two MitraClip XTR devices; one patient had a single-leaflet detachment of one of the clips with no change in regurgitation or clip position on follow-up. All patients had significant reduction of AVVR and improvement in NYHA functional class. Percutaneous atrioventricular valve repair in adults with CHD is feasible with the MitraClip but requires significant preprocedural planning and a multidisciplinary team that combines CHD and interventional therapeutic expertise.

Interstage management of pulmonary blood flow after the Norwood procedure with right ventricle-to-pulmonary artery conduit.

Our goal was to assess the efficacy of managing pulmonary blood flow from the Norwood procedure with a right ventricle-to-pulmonary artery (RV-PA) conduit until stage 2 palliation (S2P). Among 48 consecutive patients undergoing the Norwood procedure between 2008 and 2018, 40 (83.3%) patients who survived to discharge were included in this study. The primary diagnosis was hypoplastic left heart syndrome in 28 (70%) patients and hypoplastic left heart syndrome variant in 12 (30%) patients. All patients received bilateral pulmonary artery banding. The median age and weight at the time of the Norwood procedure were 41 (25th-75th percentiles: 27-89) days and 3.2 (2.7-3.9) kg, respectively. In keeping with institutional strategy, S2P was undertaken when body weight exceeded 5.0 kg, and normal gross motor development was confirmed. The RV-PA conduit was clipped in 28 (70%) patients during the perioperative period of the Norwood procedure, then partial unclipping was performed in 8 (20%) patients and full unclipping was performed in 20 (50%) patients. Before S2P, the median pulmonary-to-systemic blood flow ratio was 1.0 (0.7-1.3). The median age and weight at the time of S2P were 10.7 (9.0-12.9) months and 6.3 (5.5-7.1) kg, respectively. The survival rate 5 years after Norwood discharge was 85.3%. Pre-S2P pulmonary-to-systemic blood flow ratio was linearly correlated with greater interstage changes in systemic atrioventricular valve regurgitation (R2 = 0.223, P = 0.004). Interstage management of pulmonary blood flow by RV-PA conduit clipping and gradual unclipping provided good interstage outcomes. The median pulmonary-to-systemic blood flow ratio could be controlled to 1.0 at pre-S2P catheter examination.