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.

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.

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.

"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.

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.

Large collateral from the right coronary artery in a case of coarctation of the aorta with bilateral subclavian artery aneurysms in an adult.

Here, we present a case of a 26-year-old gentleman who presented with a rare combination of bilateral huge subclavian artery aneurysms, coarctation of the aorta with a large coronary artery collateral and a bovine aortic arch. Various mediastinal collaterals have been described in previous cases of coarctation of the aorta, but the association of coronary collaterals is a rare occurrence. To the best of our knowledge, this case has not been reported in literature yet.

A six-chambered heart: cor triatriatum sinister with double-chambered right ventricle in association with ventricular septal defect.

BACKGROUND: Cor triatriatum has been described as a heart with three atria in which the left atrium (cor triatriatum sinistrum) or right atrium (cor triatriatum dextrum) is divided into two compartments by a fold of tissue, a membrane, or a fibromuscular band. Double-chambered right ventricle, on the other hand, is identified by the presence of an anomalous muscle bundle dividing the right ventricle into two chambers. CASE PRESENTATION: Here, we describe the case of a child who had a combination of both of these rare entities, effectively creating a heart with six chambers. The child underwent a successful intracardiac repair. CONCLUSIONS: The association of CTS with DCRV forming a "6-chambered heart" is extremely rare. Awareness of its existence and accurate preoperative diagnosis has important implications in its surgical repair with all the components of this disease spectrum, further increasing the complexity of a successful surgical repair.

Assessment of factors affecting short-term pulmonary functions following cardiac surgery: A prospective observational study.

BACKGROUND: Pulmonary complications after cardiac surgery are very common and lead to an increased incidence of post-operative morbidity and mortality. Several factors, either modifiable or non-modifiable, may contribute to the associated unfavorable consequences related to pulmonary function. This study was aimed to investigate the degree of alteration and factors influencing pulmonary function (forced expiratory volume in one second (FEV1) and forced vital capacity), on third, fifth, and seventh post-operative days following cardiac surgery. METHODS: This study was executed in 71 patients who underwent on-pump cardiac surgery. Pulmonary function was assessed before surgery and on the third, fifth, and seventh post-operative days. Data including surgical details, information about risk factors, and assessment of pulmonary function were obtained. RESULTS: The FEV1 and forced vital capacity were significantly impaired on post-operative days 3, 5, and 7 compared to pre-operative values. The reduction in FEV1 was 41%, 29%, and 16% and in forced vital capacity was 42%, 29%, and 19% consecutively on post-operative days 3, 5, and 7. Multivariate analysis was done to detect the factors influencing post-operative FEV1 and forced vital capacity. DISCUSSION: This study observed a significant impairment in FEV1 and forced vital capacity, which did not completely recover by the seventh post-operative day. Different factors affecting post-operative FEV1 and forced vital capacity were pre-operative FEV1, age ≥60, less body surface area, lower pre-operative chest expansion at the axillary level, and having more duration of cardiopulmonary bypass during surgery. Presence of these factors enhances the chance of developing post-operative pulmonary complications.

Fetal cardiac intervention and fetal cardiac surgery: where are we in 21st century?

Fetal cardiac intervention is an in-utero cardiac procedure done in fetuses with heart diseases like severe aortic stenosis with evolving hypoplastic left heart syndrome, hypoplastic left heart syndrome with an intact or restricted atrial septum, pulmonary atresia with an intact ventricular septum, fetal heart block obstructed total anomalous pulmonary venous return, pericardial collection. The successful biventricular repair can be done in postnatal life after aortic or pulmonary valvuloplasty. Fetal bypass is very challenging because of different physiology. Low prime volume with the high flow can be used to prevent an inflammatory response.

A single coronary artery with left circumflex artery crossing right ventricular outflow tract in tetralogy of Fallot with absent left pulmonary artery.

Tetralogy of Fallot (TOF) with unilateral absence of pulmonary artery and the anomalous coronary artery is a rare combination. Detailed preoperative evaluation of coronary artery anatomy is must to prevent injury to the major vessels crossing right ventricular outflow tract. We report a rare association of single coronary artery with left circumflex artery crossing right ventricular outflow tract close to the pulmonary annulus in tetralogy of Fallot with absent left pulmonary artery in 11-year-old girl. Though there is a great diversity of coronary anomalies in tetralogy of Fallot, the prepulmonic course of left circumflex artery crossing the right ventricular outflow tract (RVOT) close to the pulmonary annulus has rarely been described in the literature. The patient underwent successful primary single lung intracardiac repair. Right ventricular outflow tract obstruction was treated by handmade valved pericardial autologous conduit and release of the tethering of hypoplastic native unicuspid pulmonary valve leaflet maintaining its integrity.

Short-term outcome of polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of right ventricular outflow tract in tetralogy of fallot: a randomized controlled trial

Abstract Introduction: Reconstruction of right ventricular outflow tract during primary repair of tetralogy of Fallot often requires the placement of a transannular patch which results in pulmonary regurgitation (PR). We compared the short-term outcomes of bicuspid polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of the right ventricular outflow tract. Methods: Thirty consecutive patients undergoing primary repair of tetralogy of Fallot were randomly allocated to two groups - polytetrafluoroethylene valve (PTFEV) group (n=15) and transannular pericardial patch (TAP) group (n=15). The two groups had similar preoperative demographic characteristics. We compared the short-term clinical and echocardiographic outcomes between these groups. The transthoracic echocardiographic follow-up was performed at one week, one month and six months after surgery. Results: The PTFEV group had significantly lower central venous pressure in the immediate postoperative period compared to the TAP group (7.60±2.06 vs. 10.13±1.73, P=0.002). Extubation time was significantly shorter in the PTFEV group compared to the TAP group (12.93±7.55 hrs vs. 22.23±15.11 hrs, P=0.04). PR in the PTFEV group was absent in five patients at 24 hours post-surgery. At the study endpoint, PR was absent in six, trivial in one and mild in eight patients in the PTFEV group compared to TAP group, where all 15 patients had severe PR. Conclusion: The bicuspid polytetrafluoroethylene membrane valves significantly decrease the central venous pressure in the immediate postoperative period, facilitate early extubation and, thus, prevent ventilator-related comorbidities. They achieve a high degree of pulmonary competence and do not increase the right ventricular outflow tract gradient in short-term follow-up.

Short-term outcome of Polytetrafluoroethylene Membrane Valve versus Transannular Pericardial patch Reconstruction of Right Ventricular Outflow Tract in Tetralogy of Fallot: a Randomized Controlled Trial.

INTRODUCTION: Reconstruction of right ventricular outflow tract during primary repair of tetralogy of Fallot often requires the placement of a transannular patch which results in pulmonary regurgitation (PR). We compared the short-term outcomes of bicuspid polytetrafluoroethylene membrane valve versus transannular pericardial patch reconstruction of the right ventricular outflow tract. METHODS: Thirty consecutive patients undergoing primary repair of tetralogy of Fallot were randomly allocated to two groups - polytetrafluoroethylene valve (PTFEV) group (n=15) and transannular pericardial patch (TAP) group (n=15). The two groups had similar preoperative demographic characteristics. We compared the short-term clinical and echocardiographic outcomes between these groups. The transthoracic echocardiographic follow-up was performed at one week, one month and six months after surgery. RESULTS: The PTFEV group had significantly lower central venous pressure in the immediate postoperative period compared to the TAP group (7.60±2.06 vs. 10.13±1.73, P=0.002). Extubation time was significantly shorter in the PTFEV group compared to the TAP group (12.93±7.55 hrs vs. 22.23±15.11 hrs, P=0.04). PR in the PTFEV group was absent in five patients at 24 hours post-surgery. At the study endpoint, PR was absent in six, trivial in one and mild in eight patients in the PTFEV group compared to TAP group, where all 15 patients had severe PR. CONCLUSION: The bicuspid polytetrafluoroethylene membrane valves significantly decrease the central venous pressure in the immediate postoperative period, facilitate early extubation and, thus, prevent ventilator-related comorbidities. They achieve a high degree of pulmonary competence and do not increase the right ventricular outflow tract gradient in short-term follow-up.

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.

Chronic constrictive pericarditis complicated with huge right atrial thrombus in a child with abdominal tuberculosis: a rare life-threatening condition.

Chronic constrictive pericarditis (CCP) is the most common pericardial pathology. CCP complicating with intracardiac thrombus is a rare entity, the detection of thrombus preoperatively is life-saving in avoiding the risk of pulmonary thromboembolism during anterior pericardiectomy. Transesophageal echocardiography has been shown to have better sensitivity in detecting atrial thrombus than transthoracic echocardiography. Surgical removal of right atrial thrombus under cardiopulmonary bypass should be considered.

Pulmonary hydatid cyst: Review of literature.

Echinococcosis is a rare infectious disease in human being that occurs by the larval stages of taeniid cestodes of the genus Echinococcus. Human cystic echinococcosis is the most common presentation. The liver is the most common site of echinococcal cyst, followed by the lungs. The symptoms of lung infestation lead to sudden onset of chest pain, cough, fever, and hemoptysis after a cyst rupture. The diagnosis is confirmed by radiology supplemented with serology. Chest X-ray and computer tomography of chest is the principal investigation for pulmonary hydatid cyst (PHC). The treatment of PHCs is either pharmacotherapy and/or surgery. Surgical intervention is the most preferred treatment of choice; pharmacotherapy is useful in selected patients. Pharmacotherapy includes oral administration of benzimidazoles group of drugs like mebendazole or albendazole.

A case of traumatic thoracic aorta rupture - A life threatening emergency.

Traumatic aortic transection is a life threatening emergency where there is a near-complete tear through all the layers of the aorta due to trauma. This condition is most often lethal and requires immediate medical attention. Symptoms of an aortic rupture may include severe chest pain, back pain, abdominal pain and signs of external chest injury. Treatment should be prompt in hemodynamically unstable patient in the form of endovascular or open surgical technique. We present a twenty nine year old male with aortic transection following motor vehicle accident where an interposition tube graft was placed after trimming the lacerated segments of the aorta under cardiopulmonary bypass. The patient is doing well with two years of follow up at our institution.

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.

Surgical outcome of isolated congenital supravalvular pulmonary stenosis: a case series.

BACKGROUND: Supravalvular stenosis of main pulmonary artery is a rare anomaly characterized by the presence of constriction band just above the pulmonary valve. It is mostly acquired after intervention on the pulmonary trunk or less commonly is congenital in origin associated with complex congenital cardiac malformations and very rarely can present as an isolated native congenital supravalvular pulmonary stenosis (SPS). CASE SUMMARY: We present a series of four cases of isolated congenital SPS who underwent surgical correction at our tertiary care institute over 8 years. Mean age of the patients was 2.25 ± 0.96 years with all of them being males. Mean peak systolic gradient across the stenosis was 82 ± 21.48 mmHg ranging from 60 mmHg to 110 mmHg. There was no early and medium-term mortality with 100% survival at mean follow-up of 31 months (range 7-85 months). DISCUSSION: Surgical correction of congenital SPS carries excellent early and mid-term results with almost no mortality and very low risk of re-intervention for restenosis.