Early and mid-term outcomes of double-chambered right ventricle repair: An 8-year experience.

BACKGROUND/AIM: Double-chambered right ventricle is a rare and progressive condition that is characterised by obstruction of the right ventricular tract. Double-chambered right ventricle is usually associated with ventricular septal defect. Early surgical intervention is recommended in patients with these defects. Based on this background, the present study aimed to review early and midterm outcomes of primary repair after double-chambered right ventricle. METHODS: Between January 2014 and June 2021, 64 patients with a mean age of 13.42 ± 12.31 years underwent surgical repair for double-chambered right ventricle. The clinical outcomes of these patients were reviewed and assessed retrospectively. RESULTS: An associated ventricular septal defect was present in all the recruited patients; 48 (75%) patients of sub-arterial type, 15 (23.4%) of perimembranous, and 1 (1.6%) patient of muscular type. The patients were followed up for a mean period of 46.73 ± 27.37 months. During their follow-up, a significant decrease in the mean pressure gradient from 62.33 ± 5.52 mmHg preoperatively to 15.73 ± 2.94 mmHg postoperatively was observed (p < 0.001). Notably, there were no hospital deaths. CONCLUSIONS: The development of double-chambered right ventricle in association with ventricular septal defect results in an increased pressure gradient within the right ventricle. The defect needs correction in a timely manner. In our experience, the surgical correction of double-chambered right ventricle is safe and shows excellent early and mid-term results.

Utility of portal vein pulsatility fraction in patients undergoing corrective surgery for tetralogy of Fallot.

BACKGROUND: Right ventricle dysfunction is common after corrective surgery for tetralogy of Fallot and is associated with significant morbidity and mortality. We aimed to determine whether an increased portal vein pulsatility fraction (PVPF) was associated with worse clinical outcomes. METHODS: In a prospective, observational, single-centre study, PVPF and other commonly used parameters of right ventricle function were assessed in patients of all ages undergoing corrective surgery for tetralogy of Fallot intraoperatively, with transesophageal echocardiography, before and after bypass, and post-operatively, with transthoracic echocardiography, at days 1, 2, at extubation, and at ICU discharge. The correlation was tested between PVPF and mechanical ventilation duration, prolonged ICU stay, mortality, and right ventricle function. RESULTS: The study included 52 patients, and mortality was in 3 patients. PVPF measurement was feasible in 96% of the examinations. PVPF in the immediate post-operative period had sensitivity of 73.3% and a specificity of 74.3% in predicting the occurrence of the composite outcome of prolonged mechanical ventilation, ICU stay, or mortality. There was a moderate negative correlation of PVPF with right ventricle fractional area change and right ventricle global longitudinal strain (r = -0.577, p < 0.001 and r = 0.465, p < 0.001, respectively) and a strong positive correlation with abnormal hepatic vein waveform (rho = 0.749, p < 0.001). CONCLUSION: PVPF is an easily obtainable bedside parameter to assess right ventricular dysfunction and predict prolonged mechanical ventilation, prolonged ICU stay, and mortality.

Surgical outcomes of double-orifice mitral valve repair in patients with atrioventricular canal defects: a systematic review and meta-analysis.

INTRODUCTION: Double-orifice mitral valve or left atrioventricular valve is a rare congenital cardiac anomaly that may be associated with an atrioventricular septal defect. The surgical management of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect is highly challenging with acceptable clinical outcomes. This meta-analysis is aimed to evaluate the surgical outcomes of double-orifice mitral valve/double-orifice left atrioventricular valve repair in patients with atrioventricular septal defect. METHODS AND RESULTS: A total of eight studies were retrieved from the literature by searching through PubMed, Google Scholar, Embase, and Cochrane databases. Using Bayesian hierarchical models, we estimated the pooled proportion of incidence of double-orifice mitral valve/double-orifice left atrioventricular valve with atrioventricular septal defect as 4.88% in patients who underwent surgical repair (7 studies; 3295 patients; 95% credible interval [CI] 4.2-5.7%). As compared to pre-operative regurgitation, the pooled proportions of post-operative regurgitation were significantly low in patients with moderate status: 5.1 versus 26.39% and severe status: 5.7 versus 29.38% [8 studies; 171 patients]. Moreover, the heterogeneity test revealed consistency in the data (p < 0.05). Lastly, the pooled estimated proportions of early and late mortality following surgical interventions were low, that is, 5 and 7.4%, respectively. CONCLUSION: The surgical management of moderate to severe regurgitation showed corrective benefits post-operatively and was associated with low incidence of early mortality and re-operation.

Utility of Lung Ultrasound in the Estimation of Extravascular Lung Water in a Pediatric Population-A Prospective Observational Study.

OBJECTIVE: Lung ultrasound (LUS) is a promising bedside modality for the estimation of extravascular lung water index (EVLWI), but has not been validated against objective measures in children. This study aimed to investigate the correlation of LUS B-line scoring with EVLWI, thresholds indicating elevated EVLWI, and its outcome following pediatric cardiac surgery. DESIGN: Prospective observational study. SETTING: Cardiothoracic surgical intensive care unit in a tertiary care teaching hospital. PARTICIPANTS: Children younger than 12 years undergoing elective complete surgical correction of cyanotic or acyanotic congenital heart disease (Aristotle score ≤9), excluding neonates, those weighing <3.5 kg, and those with thoracic deformities, pulmonary pathology, and hemodynamic instability. INTERVENTIONS: Extravascular lung water index measurement by transpulmonary thermodilution, along with concurrent LUS B-line and Chest-X ray (CXR) scoring. MEASUREMENTS AND MAIN RESULTS: LUS B-line score had a moderate correlation with EVLWI (Pearson's correlation coefficient 0.57; 95% CI 0.44-0.69). LUS B-line scores showed acceptable discrimination only for higher thresholds of EVLWI (sensitivity 82% and 79%, respectively, for EVLWI >20 mL/kg v sensitivity and specificity 57% and 80% for EVLWI >10 mL/kg). Age, body surface area, vasoactive-inotropic score (VIS), chest X-ray score, and EVLWI but not LUS B-line score were significant predictors for duration of mechanical ventilation in this cohort. CONCLUSIONS: LUS B-line scoring has limited utility in semiquantitative estimation of EVLWI at lower thresholds of EVLWI in pediatric cardiac surgical patients. It may have better discrimination and acceptable sensitivity and specificity at higher thresholds of EVLWI. Contrasting with multiple reports of clinical utility, these results call for wider evaluation of LUS and its clinical modifiers like age, pathology, and pretest probability in estimation of EVLWI.

Utility of Transfontanelle Ultrasound in Predicting the Fluid Responsiveness in Children After Repair of Congenital Heart Diseases: A Prospective Observational Study.

OBJECTIVES: Postoperative fluid management in children undergoing cardiac surgery requires a balanced optimization. The blood flow velocity variation in the internal carotid artery (ΔICA) measured through transfontanelle ultrasound has been shown to predict fluid responsiveness during cardiac surgery. It may provide an excellent window to study fluid responsiveness in infants during the postoperative period when the intensivist is faced with the challenges of poor echocardiographic window. The authors aimed to observe the correlation between ΔICA measured on transfontanelle ultrasound with the respiratory variability of peak aortic blood flow velocity (ΔVpeak) on transthoracic echocardiography as a marker of fluid responsiveness in infants on mechanical ventilation during the postoperative period after cardiac surgery. DESIGN: A prospective observational study. SETTING: The postcardiac surgery intensive care unit (ICU) of a tertiary care hospital. PARTICIPANTS: Thirty infants undergoing congenital cardiac surgery. INTERVENTIONS: A fluid bolus of 10 mL/kg administered over 10 minutes at 1 and 6 hours after ICU admission. Patients were categorized into fluid responders and nonresponders based on >15% increase in the indexed stroke volume. MEASUREMENTS AND MAIN RESULTS: A total of 50 fluid boluses were administered in 30 patients. Among these, 22 (73.33%) were responders and 8 (26.67%) were nonresponders. There was moderate correlation between ΔICA and peak aortic blood flow velocity variation (ΔVpeak) (r = 0.59, p ≤ 0.001). The ΔVpeak >14.74% had 68% sensitivity and 75% specificity to predict fluid responsiveness (area under the receiver operating characteristic [AUROC], 0.749; p = 0.001; positive likelihood ratio, 2.71; negative likelihood ratio, 0.43). The ΔICA >9.85% could predict fluid responsiveness in infants (AUROC, 0.728; p = 0.003; 75% sensitivity; 60% specificity; positive likelihood ratio, 1.85; negative likelihood ratio, 0.42). Among children younger than 6 months, ΔICA >9.85% was a better predictor of fluid responsiveness (AUROC, 0.889; p = 0.009) than ΔVpeak >15% (AUROC, 0.778; p = 0.061). CONCLUSION: The ICA variability >9.85% measured via transfontanelle ultrasound is a good predictor of fluid responsiveness in infants, especially those younger than 6 months on mechanical ventilation after cardiac surgery.

"Y" incision and rectangular patch enlargement of aortic annulus and valve reconstruction with autologous pericardium in congenital aortic stenosis.

Surgical aortic valve replacement in children who have undergone prior balloon or surgical valvuloplasty or both is a formidable challenge. The aortic annulus is small, there is no ideal prosthesis and lifelong anticoagulation is highly undesirable. A "Y" incision and rectangular patch enlargement of the aortic annulus introduced by Dr. Bo Yang in 2020 combined with aortic valve reconstruction introduced by Dr. Shigeyuki Ozaki in 2011, is feasible, as described in this case.

Surgical repair of ruptured sinus of Valsalva aneurysm: 13-year single center experience.

BACKGROUND AND AIM: Untreated ruptured sinus of Valsalva aneurysms ultimately develop into heart failure, thereby affecting patients' survival. We retrospectively analyzed our 13-year experience of the surgical repair for ruptured sinus of Valsalva aneurysm to study the optimal surgical strategy, operative risk and long-term surgical outcome. METHODS: Twenty-six patients underwent surgical repair of ruptured sinus of Valsalva aneurysm from January 2008 to February 2020. Follow-up data were obtained from the outpatient department records and telephone calls. RESULTS: Patch closure of ruptured sinus of Valsalva aneurysm was done in all the 26 patients, most often through the transaortic (69%) and dual-chamber approach (23%). Aortic valve repair was done in one patient while seven patients underwent aortic valve replacement for associated significant aortic regurgitation. There was one in-hospital mortality because of noncardiac cause. The median duration of postoperative hospital stay was 8 days (range, 6-11 days). Follow-up data were available for 89% (23/26) patients. The mean follow-up period was 69 ± 43 months (range, 7-147 months). All survivors were in New York Heart Association functional Class I or II. There was no late death. One patient required rehospitalization for recurrent ruptured sinus of Valsalva aneurysm. There was no recurrent or new-onset significant aortic regurgitation and prosthesis-related complications in late follow-up. CONCLUSION: Surgical repair for ruptured sinus of Valsalva aneurysm carries an acceptable low operative risk and excellent long-term outcome. Though high-risk population, an early diagnosis and optimal surgical approach can prevent worsening of symptoms and consequent heart failure.

Mixed type total anomalous pulmonary venous connection: Early results and midterm outcomes of surgical correction.

BACKGROUND AND AIM OF THE STUDY: The morphological heterogeneity of anomalous pulmonary venous drainage in mixed type total anomalous pulmonary venous connection (TAPVC) has important implications in preoperative diagnosis and surgical repair resulting in high mortality in these patients. METHODS: A retrospective review of 14 patients with mixed type TAPVC undergoing biventricular repair between January 2012 and December 2019 was conducted. A descriptive analysis was done, highlighting the anatomic variation, diagnostic and surgical approach, and surgical outcomes in these patients. RESULTS: The most common anatomic pattern was "3 by 1" (79%) followed by "2 by 2" (21%). The correct diagnosis by transthoracic echocardiography was made in 10 (71%) of the 14 patients. In contrast, preoperative computed tomographic (CT) angiography was performed in 10 patients and correct diagnosis was obtained in 8 (80%) of them. Pulmonary venous obstruction was seen in one patient before surgery. The in-hospital mortality was 14% (2/14). Four patients had pulmonary hypertensive crisis in the postoperative period. The average follow-up was 54 ± 27 months (range: 17-98 months) after surgical repair, and all surviving patients were asymptomatic. There was no late death. No clinically apparent sequelae were seen in six patients in whom isolated left superior pulmonary vein drainage was left uncorrected. CONCLUSION: An accurate diagnosis of anatomic pattern in mixed type TAPVC can be difficult to establish in all the patients before surgery. Detailed intraoperative assessment, individualized surgical approach, and aggressive perioperative management may reduce surgical mortality. Operative survivors have good midterm outcome.

Implications of left atrial enlargement and patent foramen ovale creation on early surgical outcomes in patients with total anomalous pulmonary venous connection.

INTRODUCTION: In patients with total anomalous pulmonary venous connection (TAPVC), left atrium (LA) is small and suprasystemic pulmonary artery (PA) pressures may be present in some patients. In our study, we studied the relationship between surgical LA enlargement and patent foramen ovale (PFO) creation separately on the outcomes of patients with TAPVC. MATERIALS AND METHODS: Out of the 130 patients operated in our institute between January 2014 and December 2020, LA was enlarged in 60 patients. LA enlargement was done using a larger patch for atrial septal defect (ASD) closure. Thus, the LA volume was increased by shifting the patch towards the right atrium (RA). Suprasystemic or high PA pressures were present in 60 patients. In 33 patients, PFO was created. Early surgical outcomes were determined on the basis of vasoactive inotropic score (VIS), hours of ventilation, hours of inotropic support, intensive care unit (ICU) stay, and hospital stay. RESULT: Between the LA enlarged and nonenlarged group there was statistically significant less VIS score (18 [13-27.5] vs. 24 [18-30], p value .019), hours of ventilation (23 [16-46.5] vs. 26 [18-60], p value .039), hours of inotropic support (45.5 [30-72] vs. 55 [38-84], p value .038), and ICU stay (7 [5-9] vs. 8 [7-10] p value .0352) and statistically nonsignificant less hospital stay (11.5 [9-13] vs. 12 [9-14], p value .424). In patients with preoperative suprasystemic or high PA pressures, there was a statistically significant less VIS score (16 [11-23.5] vs. 18 [13-25], p value .044), hours of ventilation (20 [14-37] vs. 22 [18-39], p value .038), hours of inotropic support (34 [29.5-71] vs. 38 [30-78], p value .042), and hospital stay (9 [5-12] vs. 11 [9-14], p value .038) and statistically nonsignificant less ICU stay (7 [5.5-9] vs. 7 [6-9], p value .886) in the group with a PFO with respect to the other group in which no PFO was created. CONCLUSION: In patients with TAPVC, LA can be enlarged by using a large ASD patch and thus shifting the septum towards RA. Early surgical outcomes were improved with LA enlargement. In patients with suprasystemic or high PA pressures, leaving a PFO improved the postoperative outcomes.

Congenital right coronary artery aneurysm with fistula to right ventricle associated with isolated pulmonary valvular stenosis.

Congenital aneurysmal dilatation of coronary artery with coronary cameral fistula is rare in childhood. We report an even rarer association of congenital right coronary artery aneurysm and right coronary artery to right ventricle fistula with bicuspid pulmonary valve stenosis and an intact ventricular septum in a 3-year-old child.

Delayed embolization of ductal occluder device into the aorta with near-complete obstruction of the aorta and critical cardiac decompensation.

Delayed embolization of ductal occluder device into the aorta after transcatheter closure of a patent ductus arteriosus (PDA) with almost complete obstruction of the aorta is extremely rare. Our patient had delayed migration of a ductal occluder device into the descending thoracic aorta (DTA) 6 months after its deployment. Because of critical biventricular dysfunction, urgent surgical removal of the device from the descending aorta was done via left posterolateral thoracotomy without using cardiopulmonary bypass. PDA was not closed because of doubt about the reversibility of pulmonary artery hypertension and severe right ventricular dysfunction so that it can act as an outlet to the decompensated right ventricle. The patient made an uneventful recovery. The patient was started on pulmonary vasodilators and is planned for right heart catheterization study after 2 months to check for the operability of PDA.

Absent right superior vena cava with left superior vena cava draining into unroofed coronary sinus with common atrium in a child with an atrioventricular septal defect.

The association of absent right superior vena cava and persistent left superior vena cava draining into unroofed coronary sinus with common atrium and the atrioventricular septal defect is an extremely rare form of the congenital cardiac disorder with only one case reported so far, hence, can be missed preoperatively if not carefully looked for. Failure to detect absent right superior vena cava beforehand may otherwise pose difficulties in carrying out invasive surgical or medical interventions.

Pulmonary valve neocuspidisation with glutaraldehyde-treated autologous pericardium-A novel technique in pulmonary valve endocarditis.

Infective endocarditis (IE) is a serious condition leading to heart failure, persistent sepsis. The management of IE involving valve is mainly excision of the infected valve and replacement with a heart valve; which are also at the risk of prosthetic valve endocarditis. Hence repair of the valve with autologous pericardium is much more physiological. We had a 20-year-old male presented with features of heart failure and high-grade fever not responding to optimum medical management. Two-dimensional echocardiogram revealed vegetation on pulmonary valve cusps with the erosion of the left and right cusps. Neo cusps with autologous pericardium offered good hemodynamics with trivial regurgitation. The patient is doing well with normal pulmonary valve function 3 months after surgery. This technique is reliable, economic, and easily reproducible.

Benefits of perioperative sildenafil therapy in children with a ventricular septal defect with pulmonary artery hypertension on early surgical outcomes.

OBJECTIVES: Pulmonary hypertension is a common association in children with nonrestrictive ventricular septal defect. It increases perioperative mortality and morbidity. Oral sildenafil is an effective pulmonary vasodilator. In this study, we assessed effects of perioperative oral sildenafil therapy on pulmonary artery pressure and early surgical outcomes. METHODS: This was a single centre, prospective randomized control study. Thirty children with nonrestrictive ventricular septal defects with pulmonary hypertension were divided into two groups. In the sildenafil group (n = 15, mean age 23.3 months), oral sildenafil was administered two weeks before surgery. In the control group (n = 15, mean age 36 months), preoperative sildenafil was not given. Sildenafil was continued postoperatively in both groups, provided the postoperative pulmonary artery pressure was over 50% of systemic pressure. RESULTS: There was no perioperative mortality, pulmonary hypertensive crisis and there were no intolerable side effects related to sildenafil in either group. Mean pulmonary artery pressure showed a reduction in both groups. Sildenafil group showed statistically significant improvement in duration of cardiopulmonary bypass (100.27 ± 21.09 min vs. 125.40 ± 26.83 min, p = .008), mechanical ventilation requirement (22.79 ± 17.13 h vs. 30.53 ± 13.05 h; p = .04), epinephrine requirement (22% patients vs. 48% patients; p = .03) and hospital stay (6.13 ± 1.40 days vs. 7.53 ± 1.92 days; p = .05). CONCLUSION: Oral Sildenafil therapy is an inexpensive and well-tolerated method for reducing pulmonary hypertension secondary to non-restrictive ventricular septal defect. It has noteworthy advantages regarding early surgical outcomes like reduced cardiopulmonary bypass time, improved mechanical ventilation time, lower inotrope requirement and shorter hospital stay if used preoperatively in select patient population.

Surgical outcome in aortopulmonary window beyond the neonatal period.

OBJECTIVE: Aortopulmonary window (APW) is a rare congenital cardiac defect accounting for 0.1% to 0.2% of all congenital cardiac defects. We here present the current midterm outcome of surgical repair of APW in patients more than 3 months of age. METHODS: The retrospective study was conducted to identify all the patients more than 3 months of age at presentation who underwent surgical repair of APW between June 2010 and August 2018 at our tertiary care institute and their outcome was analyzed. RESULTS: We found 14 patients of APW operated at the age of more than 3 months over a period of 8 years. Mean age of the cohort was 2.29 ± 2.96 years ranging from 3 months to 10 years with 57.14% being males. There were 11 (78.57%) patients with isolated APW and 3 (21.43%) had associated cardiac defects including tetralogy of Fallot (n = 1), ventricular septal defect (n = 1), subaortic membrane causing subaortic stenosis (n = 1), and one had extracardiac malformations. Two patients had type I, nine had type II, and three had type III APW as per Jacobs' classification. The mean size of the defect was 14.14 ± 4.33 mm. Mean duration of mechanical ventilation was 26.91 ± 16.65 hours (range, 12.25-67 hours). There was one in-hospital mortality and no late mortality over a mean follow-up of 3.06 ± 2.19 years. None of the patients required any kind of reintervention. CONCLUSION: Good results can be obtained even on late presentation with adequate perioperative care of the patients with the reversible pulmonary hypertensive disease.

Surgical experience with cor triatriatum repair beyond infancy.

BACKGROUND: Cor triatriatum is an exceedingly rare congenital heart defect which can present at any age depending upon the presence of associated cardiac anomalies and the size of communication between the common chamber and the left atrium. Our aim is to present 10 years surgical outcome of cor triatriatum repair in patients who present beyond infancy. METHODS: On going through our institute's surgical database from January 2009 to June 2019 for cor triatriatum, 14 patients were found to have undergone the surgical correction. Their demographic profile and midterm outcome in terms of mortality, functional status, and cardiac function was assessed. RESULTS: There were total 14 patients with slight female predominance (57.14%) who underwent cor triatriatum surgical repair. The mean age at the time of surgery was 12.14 ± 9.97 years ranging from 1 to 29 years. Associated congenital cardiac defects were seen in 12 (85.71%) patients with atrial septal defect being the most common. Partial anomalous pulmonary venous connection was seen in 4 (28.57%) patients. Eight (57.14%) patients had severe pulmonary hypertension preoperatively. All but two patients showed marked reduction in pulmonary artery pressures postoperatively. All the patients underwent excision of the membrane and repair of associated cardiac defects simultaneously. There was no early or midterm mortality or any reintervention over a mean follow-up period of 58.06 ± 30.73 months (range 20-120 months). CONCLUSION: The early and midterm surgical outcome of cor triatriatum is excellent even in patients who present beyond infancy with reversible pulmonary artery hypertension.

Perioperative echocardiography-derived right ventricle function parameters and early outcomes after tetralogy of Fallot repair in mid-childhood: a single-center, prospective observational study.

BACKGROUND: Right ventricular (RV) function alterations are invariably present in all patients after tetralogy of Fallot (TOF) repair. Unlike the developed world where most of the patients with TOF are corrected in infancy, average age of presentation and thus surgery for these patients in the developing world may be higher. We aimed to study the correlation between RV function parameters such as tricuspid annular peak systolic excursion (TAPSE), fractional area change (FAC), and tricuspid annular peak systolic velocity (S') with early outcome variables after intracardiac repair for TOF. MATERIALS AND METHODS: Fifty patients with a preoperative diagnosis of tetralogy of Fallot scheduled for corrective surgery were included in this single-center, prospective observational study. A preoperative transthoracic echocardiogram was performed to measure RV function parameters (FAC0, TAPSE0, S'0). Transthoracic echocardiography was repeated postoperatively to measure FAC1, TAPSE1, S'1 (day 1) and FAC2, TAPSE2, and S'2 (day 3). The relationship between preoperative and postoperative RV function parameters with in-hospital mortality, duration of mechanical ventilation, and intensive care unit stay was studied. RESULTS: The median age of patients was 6 years (range 1-14 years). Multiple stepwise logistic regression analysis showed RV FAC as best predictor of clinical outcome. Area under the receiver operating characteristic curve for postoperative RV function parameters, that is, FAC, TAPSE, and S' to predict early or delayed recovery was 0.944, 0.875, and 0.655, respectively. CONCLUSIONS: Among the RV function parameters studied, RV FAC best predicted the early outcome variables after TOF repair, followed by TAPSE while lateral tricuspid annular velocity S' being the least predictive.

Perioperative Follow-Up of Patients With Severe Pulmonary Artery Hypertension Secondary to Left Heart Disease: A Single Center, Prospective, Observational Study.

OBJECTIVE: A substantial portion of the Indian cardiac surgery population experiences rheumatic valve disease that progresses to severe pulmonary artery hypertension (PAH) in a few patients. Right ventricular (RV) function, particularly in the perioperative period, has been studied sparsely. The authors describe serial RV function and clinical variables in the perioperative period in patients with severe PAH secondary to left heart disease. DESIGN: Prospective, observational study. SETTING: University hospital. PARTICIPANTS: Patients with PAH. INTERVENTIONS: The study comprised consecutive patients referred for open cardiac surgery from January 2012 to June 2013 who also had an estimated right ventricular systolic pressure≥50 mmHg on referral echocardiogram. Composite echocardiographic assessment of right ventricular size and linear/two-dimensional tissue Doppler systolic function and diastolic function analysis were performed at predetermined intervals. Data from right heart catheterization, inotrope use, fluid requirements, mechanical ventilation logs, and intensive care stay also were acquired. MEASUREMENTS AND MAIN RESULTS: A complete dataset was obtained in 20 of 22 patients enrolled in the study. Serial comparison of most RV echocardiographic function variables were noted to be abnormal at baseline, deteriorating further in the immediate postoperative period and trending to a partial recovery at discharge from the intensive care unit, particularly for longitudinal assessment of the RV. Fractional area change, although abnormal, was noted to be preserved. Pulmonary artery systolic pressures registered significantly declined after intervention. The clinical course was largely uneventful. CONCLUSION: Although linear echocardiographic RV function was grossly abnormal in the perioperative period in this patient subset with PAH, there was apparent disjunction with the clinical course.

FibroScan as a surveillance marker post-cavopulmonary anastomosis for single-ventricle physiology.

Purpose: Single-ventricle physiology encompasses a group of congenital cardiac malformations with only one functional ventricle. The Fontan procedure is the final palliation of this pathway and has its complications. One of these is Fontan-associated liver disease (FALD). It is known that all patients with Fontan will have FALD, due to the physiology of the Fontan circuit, and only the severity will vary. The pathophysiology of hepatic damage in FALD is unique and not easily detectable by routine non-invasive investigations. Therefore, this study is aimed to identify if FibroScan can be used as a surveillance marker to detect and assess the progression of FALD. Methods: Patients who attended the Cardiothoracic and Vascular Surgery Outpatient Department (OPD) for follow-up post-cavopulmonary anastomosis (bidirectional Glenn and Fontan) were enrolled in this study. They underwent routine examinations and tests, and in addition a FibroScan was performed. Results: FibroScan showed that the liver stiffness measurement (LSM) was increased in all patients who had undergone Fontan and a couple of patients who had undergone bidirectional (BD) Glenn. The LSM was 12.29 (± 3.59) kPa in patients post-Fontan and 6.64 (± 4.24) kPa in patients post-BD Glenn. This raised LSM was not associated with a parallel rise in liver enzymes or other laboratory markers associated with liver function. This emphasizes the suitability of FibroScan as an early and non-invasive surveillance tool for monitoring the progression of hepatic venous congestion and FALD. Conclusion: We have found that LSM via FibroScan can effectively be a surveillance or screening test for FALD. Serial FibroScans can be used to monitor the progress of liver disease. Raised LSM may also have a role in predicting the morbidity for completion of Fontan post-BD Glenn operation. Large-scale studies are needed to validate our findings.