Right Atrial Strain in Pediatric Pulmonary Hypertension-A Prospective Observational Study.

Right ventricular (RV) afterload due to elevated pulmonary arterial (PA) pressure in pulmonary hypertension (PH) causes long-term right atrial (RA) remodeling and dysfunction. RA function has been shown to correlate with PA pressures and outcome in both adult and pediatric patients with PH. We studied the role of RA strain in estimating PA pressures in congenital heart disease (CHD)-associated PH. Children below 12 years undergoing elective repair of CHD with left-to-right shunts and echocardiographic evidence of PH were included. RA reservoir, conduit and contractile strain along with conventional measures of RV function and PA pressure were measured using transthoracic echocardiography after induction of anaesthesia. Pre-and post-repair invasive PA pressures were measured after surgical exposure. 51 children with a median age of 24 months (range 4-144 months) were included, most of whom were undergoing VSD closure. Contractile RA strain showed good correlation with pre-repair systolic PA pressure in mmHg (r = 0.59, 95%CI 0.37-0.75) or expressed as a percentage of SBP (r = 0.67, 95%CI 0.49-0.80). It also predicted persistent postoperative PH as well as pre-repair pulmonary artery acceleration time and right ventricular systolic pressure measured from tricuspid regurgitation jet. The trends of correlation observed suggest a possible prognostic role of RA strain in ACHD with PH and potential utility in its echocardiographic assessment. The observed findings merit deeper evaluation in larger cohorts.

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.

Effect of Thyroidectomy on Tracheal Remodeling and Airway Physiology in Apparently Asymptomatic Patients with Goiter: A Prospective Study.

BACKGROUND: Tracheal airflow limitation is frequently reported in patients with goiter but is severely underestimated, and studies on how goiter and its treatment affect trachea are scarce. Moreover, the choice of the optimal treatment for individual patient with asymptomatic goiter is not straightforward. Therefore, in this study we aim to investigate the effect of goiter and subsequent thyroidectomy on tracheal anatomy and change in airflow in asymptomatic patient with goiter. METHODS: Seventy patients undergoing total/hemithyroidectomy (TT/HT) from Feb 2020 to Feb 2021 satisfying inclusion criteria were enrolled in the study. Neck radiograph (NR) and forced spirometry (FS) were performed preoperatively and on postoperative day 10 and 6 weeks and 3 months. RESULTS: Out of 70 patients, 84.3% patients were female, and mean duration and weight of goiter were 54.7 months and 72.21 gm, respectively. Of 70 patients, 57 were of benign pathology. Significant improvement in tracheal compression with moderate improvement in deviation was observed after surgery. Preoperative spirometry showed significant reduction in almost all parameters. After surgery, a weak improvement was observed at postoperative day 10 and 6 weeks; however, significant improvement in FEV1, PEFR, FEV1/FEV0.5, and FEF50%/FIF50% was observed at postoperative 3 months. Patient with right sided and those with ≥ 8 mm deviation were associated with poorer pulmonary function. Weak correlation was observed between neck NR and spirometry parameters. Weight of the thyroid gland significantly correlated with ratio of MVV/FEV1. CONCLUSION: Patients with asymptomatic goiter can have significant abnormal changes in airflow as evidenced by FS and NR. Thyroidectomy is followed by gradual restoration of tracheal deviation and compression with significant improvement in pulmonary airflow.

Arterial switch operation and aortic valve replacement for transposition of great arteries in adulthood: two cases from a tertiary care centre in India.

The surgical treatment of transposition of the great arteries, ventricular septal defect, and significant left ventricular outflow tract obstruction continues to evolve. The survival of an unrepaired transposition of the great arteries into late adulthood is a rarity. Even when large intracardiac shunts are present, it remains a lethal cyanotic CHD if it is not surgically corrected soon after birth. We present our experience of two cases, both of whom underwent a single-stage arterial switch operation and an aortic valve replacement for this defect.

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.

Role of Lung Ultrasound in the Detection of Postoperative Pulmonary Complications in Pediatric Patients: A Prospective Observational Study.

OBJECTIVE: To evaluate the incremental benefit of lung ultrasound (LUS) over clinical examination and chest x-rays (CXR) together (clinico-radiologic examination) for the diagnosis of postoperative pulmonary complications (PPC). DESIGN: Prospective observational study. SETTING: Tertiary care center. PARTICIPANTS: One hundred children after corrective congenital cardiac surgery with left-to-right shunts. INTERVENTION: Participants were independently evaluated with clinico-radiologic examination by the treating team, as well as LUS by an investigator at 12, 24, 48, and 72 hours after surgery. After recording the diagnoses, the LUS findings were disclosed to the treating team and a final diagnosis was made. CXR scores and LUS scores were evaluated for their ability to predict PPC. MEASUREMENTS AND MAIN RESULTS: A total of 34 cases of PPCs were observed. Of these, 32 each were detected by clinico-radiologic examination and LUS alone. Addition of LUS improved total number of PPCs detected in the early postoperative period but not in the late postoperative period. Preoperative and early postoperative LUS scores were superior to CXR scores in predicting occurrence of PPC (area under receiver operating characteristics curve [AUROC] 0.920 v 0.732; p < 0.001 preoperatively; AUROC 0.987 v 0.858, p = 0.001 at 12 hours postoperatively). Multivariate analysis suggested LUS score as an independent predictor of PPC, and LUS score along with aortic cross-clamp time as independent predictors of duration of mechanical ventilation and intensive care unit stay. CONCLUSIONS: LUS improves identification of PPC over clinico-radiologic examination in the early postoperative period. Preoperative LUS scores have better predictive ability than CXR scores for the occurrence of PPC.

Rare branching pattern of the aortic arch in a neonate.

Here, we describe the case of a neonate who was found to have a complex pattern of branching on radiological imaging. The right common carotid artery, left common carotid artery and left subclavian artery were arising as a common single trunk from the arch of aorta with the additional presence of an aberrant right subclavian artery from the distal aortic arch on the left side and having a retroesophageal course to reach the right arm.

Surgical management of aortopulmonary window with pulmonary artery dilatation causing left main coronary compression.

Aortopulmonary window is a rare congenital heart defect. Left main coronary artery extrinsic compression by an enlarged pulmonary artery is a rare complication and a potential cause for chest pain and sudden cardiac death in patients with pulmonary hypertension. Here, we present the case of a 14-year-old boy with a large aortopulmonary window who was planned for a device closure, but during the procedure, he developed ST-T segment changes while the device was being deployed, and hence the procedure was abandoned. The boy subsequently underwent a successful surgical closure thereafter.

Successful Surgical Management of a Rare Combination of Intrapericardial Teratoma and Thymoma in an Infant.

Primary cardiac tumors are rare, accounting for <0.2% of all childhood tumors. They can be diagnosed prenatally. Intrapericardial teratoma is a rare benign tumor that presents either due to the mass effect of the tumor or secondary pericardial effusion. Thymus is an important part of the immune system in the pediatric age group. Thymic lesions are rare causes of anterior mediastinal pathology. Their occurrence in children is rarer, nevertheless knowledge about their pathologies helps in clinching the correct diagnosis. We report a case of combined intrapericardial teratoma and thymoma that has not been reported previously in the literature.

Supravalvular pulmonary stenosis 9 years after surgically created pleuro-pericardial window.

Supravalvular pulmonary stenosis acquired as a postoperative cardiac procedure complication is relatively common. However, that occurring after a surgically created pleuro-pericardial window has not been described until now, to the best of our knowledge. We present a case of acquired supravalvular pulmonary stenosis that developed 9 years after the pleuro-pericardial window creation for pyopericardium due to a constricting pericardial band. The child underwent successful surgical relief of the stenosis along with repair of the atrial and ventricular septal defects.

Ebstein's anomaly of tricuspid valve with aortic stenosis and coarctation of aorta: Successful single-stage repair of a rare adult congenital heart disease.

Ebstein's anomaly of the tricuspid valve is infrequently associated with left heart anomalies. The association of aortic stenosis in Ebstein's anomaly has been reported to be extremely rare and the association of coarctation of aorta is even rarer especially in adults. The combination of all three of these lesions is virtually unknown without any references in literature. We report here an unusual case of Ebstein's anomaly of the tricuspid valve and severe aortic stenosis with coarctation of aorta in an adult who presented to us with exertional dyspnoea in the third decade and underwent a successful single-stage intracardiac repair.

Emergency valve preserving ascending aorta replacement following iatrogenic right coronary artery dissection: A life-saving procedure.

Iatrogenic aortocoronary dissection is a rare but potentially fatal complication of coronary catheterizations. Although the incidence is comparatively low, dissection often leads to procedure failure with increased risk of myocardial infarction and death. Iatrogenic aortocoronary dissection is principally caused by disruption of intima at the ostia of the right or left coronary artery during interventional procedures and appears as luminal filling defects, the persistence of contrast or intimal tear outside the coronary lumen. We present a case of right coronary artery dissection leading to type-A aortic dissection suffered during diagnostic coronary catheterization. This required emergency supracoronary replacement of the ascending aorta with an aortic interposition tube graft and venous grafts to coronary arteries.

Atretic right superior vena cava with left superior vena cava draining directly into left atrium with absent coronary sinus in an adult with partial atrioventricular canal defect and complete heart block.

The association of atretic right superior vena cava with persistent left superior vena cava draining directly into left atrium with absent coronary sinus in atrioventricular canal defect is virtually unknown in adults with no case reported so far. Though atretic right superior vena cava with persistent left superior vena cava is an extremely rare venous anomaly seen in congenital heart disease, it has important clinical implications in cardiac surgery and interventional cardiology. Atrial arrhythmias and right bundle branch block are common with advancing age in partial atrioventricualr canal defect but complete heart block has scarcely been reported in the medical literature.

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 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.

Comparative Effect of Levosimendan and Milrinone in Cardiac Surgery Patients With Pulmonary Hypertension and Left Ventricular Dysfunction.

OBJECTIVE: To compare the effects of levosimendan with milrinone in cardiac surgical patients with pulmonary hypertension and left ventricular dysfunction. DESIGN: A prospective, randomized study. SETTING: Tertiary care teaching hospital. PARTICIPANTS: The study included patients with valvular heart disease and pulmonary artery hypertension undergoing valve surgery. INTERVENTIONS: Forty patients were allocated randomly to receive either milrinone, 50 µg/kg bolus followed by infusion at a rate of 0.5 µg/kg/min (group 1), or levosimendan, 10 µg/kg bolus followed by infusion at a rate of 0.1 µg/kg/min (group 2) for 24 hours after surgery. MEASUREMENTS AND MAIN RESULTS: Hemodynamic parameters were measured using a pulmonary artery catheter, and biventricular functions were assessed using echocardiography. Mean pulmonary artery pressures and the pulmonary vascular resistance index were comparable between the 2 groups at several time points in the intensive care unit. Biventricular function was comparable between both groups. Postcardiopulmonary bypass right ventricular systolic and diastolic functions decreased in both groups compared with baseline, whereas 6 hours postbypass left ventricular ejection fraction improved in patients with stenotic valvular lesions. Levosimendan use was associated with higher heart rate, increased cardiac index, decreased systemic vascular resistance index, and increased requirement of norepinephrine infusion compared with milrinone. CONCLUSIONS: The results of this study demonstrated that levosimendan was not clinically better than milrinone. Levosimendan therapy resulted in a greater increase in heart rate, decrease in systemic vascular resistance, and a greater need for norepinephrine than in patients who received milrinone.

Benign granular cell tumor of the lung in a child--a case report.

Amongst the lung tumors, granular cell tumor (GCT), also known as granular cell myoblastoma, or Abrikossoff is one of the rarest tumors. Herein, we present a case of a 6-year-old male child who presented with a history of cough, fever and recurrent pneumonia due to a 8 × 5 × 3 cm GCT.