Tricuspid Annular Plane Systolic Excursion (TAPSE) for the Assessment of Right Ventricular Function in Adult and Pediatric Cardiac Surgery: Modified Two-dimensional and M-mode TAPSE by Transesophageal Echocardiography Compared to M-mode TAPSE by Transthoracic Echocardiography.

OBJECTIVES: To compare transesophageal echocardiography-guided 2-dimensional and M-mode tricuspid annular plane systolic excursion (TAPSE) to transthoracic echocardiography (TTE)-guided M-mode TAPSE in terms of accuracy, interobserver, and intra-observer variability. DESIGN: A prospective, observational study. SETTING: Adult and pediatric operating rooms. PARTICIPANTS: Adult and pediatric patients (42 each) undergoing cardiac surgeries. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Modified midesophageal (Mod-ME), deep transgastric, and transgastric TAPSEs were analyzed for reliability and were compared to TTE TAPSE in both adult and pediatric groups. Modified ME TAPSE showed good method agreement with TTE TAPSE (bias = -0.97, p = 0.08 (adult); bias = 0.17, p = 0.71 [pediatric]), and showed a moderate correlation with right ventricular (RV) fractional area change (FAC) (r = 0.41, p = 0.006, [adult]; r = 0.57, p < 0.001, [pediatric]), with acceptable interobserver variability (percentage error =10.56 [adult]; 4.42 [pediatric]) and intraobserver variability (percentage error = 13.1 [adults]; 12.24 [pediatric]). Transgastric TAPSE poorly agreed with TTE TAPSE and had higher interobserver and intraobserver variability. Deep transgastric TAPSE had good method agreement with TTE TAPSE and had acceptable interobserver and intra-observer variability. CONCLUSIONS: Modified ME TAPSE is a reliable and reproducible measure of RV function before pericardiotomy in both adult and pediatric cardiac surgery. Right ventricular FAC values reflected the RV systolic function better than TAPSE after pericardiotomy. Deep transgastric TAPSE is reliable and reproducible but is less accurate than Mod-ME TAPSE.

Early and Long-Term Clinical Outcomes of Ductal Stenting Versus Surgical Aortopulmonary Shunt Among Young Infants with Duct-Dependent Pulmonary Circulation.

Surgical aortopulmonary shunting (SAPS) and ductal stenting (DS) are the main palliations in infants with cyanotic congenital heart diseases (CHD). We aimed to study the safety and efficacy of DS and to compare it with SAPS as a palliative procedure in infants with CHD and duct-dependent pulmonary circulation. Retrospective institutional clinical data review of consecutive infants aged < 3 months who underwent DS or SAPS over 5 years. The primary outcome was procedural success which was defined as event-free survival (mortality, need for re-intervention, procedural failure) at 30 days post-procedure. The secondary outcome was defined by a composite of death, major adverse cardiovascular events, or need for re-intervention at 6 months and on long-term follow-up. We included 102 infants (DS, n = 53 and SAPS, n = 49). The median age at DS and SAPS was 4 days (IQR 2.0-8.5) and 8 days (IQR 4.0-39.0), respectively. The median weight at intervention was 3.0 kg (IQR 3.0-3.0) and 3.0 kg (IQR 2.5-3.0) in the two respective arms. Tetralogy of Fallot with pulmonary atresia was the most common indication for DS and SAPS. The 30-day mortality was significantly higher in SAPS group as compared with DS group (p < 0.05). However, 30-day major adverse cardiac events (MACE) rates were similar in both groups (p = 0.29). DS was associated with shorter duration of mechanical ventilation, duration of stay in the intensive care and hospital stay than with SAPS. At 6 months, there was no significant difference in terms of mortality or event-free survival. Long-term MACE-free survival was also comparable (p = 0.13). DS is an effective and safer alternative to SAPS in infants with duct-dependent pulmonary circulation, offering reduced procedure-related mortality and morbidity than SAPS. Careful study of ductal anatomy is crucial to procedural success. However, long-term outcomes are similar in both procedures.

Three-dimensional electroanatomical mapping guided right bundle branch pacing in congenitally corrected transposition of great arteries.

AIMS: The ideal pacing strategy has been the Achilles' heel for patients with congenitally corrected transposition of great arteries (ccTGA) with bradycardia. Various pacing modalities were documented in the literature. This article describes a novel pacing strategy and its feasibility in ccTGA with an intact ventricular septum. METHODS AND RESULTS: We prospectively recruited three patients with ccTGA who presented with symptomatic complete heart block to our institute and were evaluated. All patients were planned for conduction system pacing. Those who had more than moderate or severe systemic atrioventricular regurgitation and systemic ventricular dysfunction were planned for conduction system pacing with an additional lead in the coronary sinus (CS) tributary, i.e. bundle branch pacing optimized cardiac resynchronization therapy with the intention to achieve incremental benefit. Since right bundle pacing is not described previously and in view of anatomical complexity in location, three-dimensional (3D) anatomical mapping was done with the EnSite system and later right bundle capture is identified conventionally as that of a left bundle in a normal heart. All three patients have stable lead positions and adequate thresholds at short-term follow-up. CONCLUSION: In this report, we demonstrated the feasibility of permanent physiological pacing of the systemic ventricle by capturing the right bundle with 3D anatomical mapping guidance, which results in physiological activation of the systemic ventricle.

Clinical profile and outcomes of pediatric hypertrophic cardiomyopathy in a South Indian tertiary care cardiac center: a three decade experience.

INTRODUCTION: Although much research has been done on adult hypertrophic cardiomyopathy, data on pediatric hypertrophic cardiomyopathy is still limited. METHODS AND RESULTS: The study enrolled all patients with cardiomyopathy who presented to us between 1990 to 2020 and were younger than 18 yrs. During the thirty-year study period, we identified 233 cases of pediatric cardiomyopathy. Sixty-three cases (27%) had hypertrophic cardiomyopathy. Out of the 63 HCM cases, 12% presented in the neonatal period and 37% presented in the first year of life. The median age of presentation was 7 yrs (Range 0.1-18 yrs). Sixteen patients had proven syndromic, metabolic, or genetic disease (25%). LV outflow obstruction was present in 30 patients (47%). Noonan syndrome was present in 9 of the 63 patients (14%). Dyspnea on exertion was the most common mode of presentation. Cardiac MRI was done in 28 patients, out of which 17 had late gadolinium enhancement (LGE). Mid myocardial enhancement was the most common pattern. Four patients had LGE of more than 15%. Over a mean follow-up period of 5.6 years (0.1-30 years), twenty-one were lost to follow-up (33%). Among the patients whose outcome was known, eleven died (26%), and thirty-one (73%) were alive. The 5-year survival rate of HCM patients was 82%, and the 10-year survival rate was 78%. Seven died of sudden cardiac death, three from heart failure, and one from ventricular arrhythmias. Sustained ventricular arrhythmias were seen in three patients and atrial arrhythmias in two. First-degree AV block was seen in 10 patients (15%) and bundle branch blocks (BBB) in five (8%). Eight patients required ICD or transplant (12.7%). Two patients underwent ICD for primary prevention, and one underwent PPI for distal AV conduction disease. Among the various clinical, echocardiographic, and radiological risk factors studied, only consanguinity showed a trend towards higher events of death or ventricular arrhythmias (P-value 0.08). CONCLUSION: More than one-third of our HCM cohort presented in infancy. LV outflow tract obstruction is common (47%). Mid myocardial enhancement was the most common pattern of late gadolinium enhancement. SCD was the most common cause of death. The outcome in our HCM cohort is good and similar to other population cohorts. Only Consanguinity showed a trend towards higher events of death or ventricular arrhythmias.

Heart failure in a newborn with tetralogy of Fallot: uncommon association of a common anomaly.

Heart failure in tetralogy of Fallot in the newborn period is rare and is usually due to either large aortopulmonary collaterals or absent pulmonary valve syndrome with severe pulmonary regurgitation. Pink tetralogy of Fallot and tetralogy of Fallot with disconnected pulmonary arteries from the aorta may present beyond the newborn period with heart failure when Pulmonary vascular resistance (PVR) falls. We describe the diagnostic and therapeutic pathway in a rare case of heart failure in newborn with tetralogy of Fallot.

Safety of drug-eluting stents for stenting patent arterial duct in neonates.

OBJECTIVES: The primary objective was to evaluate the trend of blood sirolimus concentrations in neonates following ductal stenting. The long-term outcomes and incidence of infections were also evaluated. METHODS: Prospective open-label observational study in a tertiary referral centre over a 1-year period. Serum sirolimus levels were estimated at 1 hour and 24hrs post-stent insertion followed by 7 days in neonates who underwent ductal stenting. The trend in sirolimus levels, incidence of infections, complications and outcomes following ductal stenting were studied. RESULTS: Seven neonates with duct-dependent pulmonary circulation underwent ductal stenting at median age of 8.5 days and weight of 2.83kg. The average stent size was 3.5±0.4 mm, and average stent length was 16.3±5.1 mm. The mean sirolimus concentrations at 1 hour, 24 hours and 7 days were 41.3±6.9ng/ml, 15.4±7.1ng/ml and 3.1±0.85ng/ml respectively. Levels fell below therapeutic range for all patients by 7 days. Three patients had sepsis or necrotising enterocolitis, but responded well to antibiotics; 1 patient had aspiration related sudden death. There were no further events at a mean follow-up of 207 days, and 4 patients underwent elective surgery at 238 ± 81 days after ductal stenting. CONCLUSIONS: This study demonstrates applicability of drug-eluting stents for ductal stenting in newborns. Drug-eluting stents with abluminal drug delivery are associated with high sirolimus levels in initial hours but rapidly taper to negligible levels within a week of implantation. Neonates with high pre-procedure likelihood of infection developed sepsis but responded well to conservative management. The patency of drug-eluting ductal stents is preserved over long-term follow-up.

Left ventricular-infundibular apical septal defect: a rare entity revisited.

Apical ventricular septal defects are a heterogeneous group of septal defects that need accurate anatomic characterisation for planning appropriate management. Left ventricular-infundibular apical septal defects are defects between the left ventricular septum and the infundibular apex of the right ventricle with distinctive morphological features. We describe two illustrative examples of this septal defect with focus on their therapeutic implications and long-term outcome.

Hemianomalous pulmonary venous drainage and type IIb coronary sinus septal defect - Utility of subxiphoid and right parasternal views.

A 3-year-old boy presented with history of recurrent respiratory tract infections in infancy. Clinically he had hemodynamically significant pre-tricuspid left-to-right shunt and no pulmonary hypertension. Transthoracic echocardiography delineated anomalous drainage of the left sided pulmonary veins to a dilated coronary sinus opening into the right atrium. Closer evaluation from the subxiphoid and right parasternal views led to the diagnosis of an associated type IIb coronary sinus septal defect.

A Comparative Study of Invasive Modalities for Evaluation of Pulmonary Arteriovenous Fistula after Bidirectional Glenn Shunt.

Development of pulmonary AV fistula (PAVF) after bidirectional glenn shunt (BDG) results in significant cyanosis, impaired exercise performance, and increased morbidity and mortality. We attempted to detect and quantify PAVF in post-BDG patients by saline contrast transesophageal echocardiography (TEE) and compare with pulmonary angiography and pulmonary vein oximetry. This was a prospective study done between 2017 and 2018. Twenty-five children who underwent BDG and planned for cardiac catheterization prior to Fontan completion were included in the study. All patients underwent pulmonary angiography, oximetry, and saline contrast TEE at the time of cardiac catheterization. Twenty-two patients had undergone unilateral BDG surgery and three were palliated by bilateral BDG. The mean oxygen saturation was 80 ± 5.2%. Thirteen patients (52%) had preserved antegrade pulmonary blood flow. Eighteen patients (72%) had PAVF by angiography and oximetry, while 19 (76%) had PAVF identified by contrast echocardiography. There was moderate correlation between the degree of pulmonary venous desaturation and grading of PAVF by contrast echocardiography. PAVF was predominantly located in the lower zones of the lungs. Higher grades of PAVF were not seen in patients with preserved antegrade flow after BDG. Angiographically detected PAVF showed a steady increase with increasing delay to cardiac catheterization from BDG. Significant reduction in systemic saturation was limited to advanced grades of PAVF in patients after BDG. Saline contrast TEE, pulmonary venous oximetry, and pulmonary angiography equally identified PAVF in patients after BDG. Prognostic utility of the same needs to be assessed by long-term follow-up of these subjects.

Stenting of the right ventricular outflow tract as an initial intervention in Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries.

OBJECTIVES: To assess the role of right ventricular outflow tract stenting as the primary intervention in Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries. BACKGROUND: The management of a subset of infants with Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries requires a staged approach including rehabilitation of diminutive native pulmonary arteries, conventionally using an aortopulmonary shunt. We share our experience of pulmonary artery rehabilitation with right ventricular outflow tract stenting. METHODS: Retrospective review of all patients with Tetralogy of Fallot with pulmonary stenosis who underwent right ventricular outflow tract stenting as primary intervention over an 8-year period. RESULTS: Ten patients underwent right ventricular outflow tract stent insertion at a median age of 61 days (interquartile range (IQR) 8.3-155 days). Median weight at stent deployment was 4.2 kg (IQR 3.2-5.7 kg). Oxygen saturations improved from a median of 79% (IQR 76-80%) to 92% (IQR 90-95%), p = 0.012. The median right and left pulmonary artery z score increased from -3.51 (IQR -4.59 to -2.80) and -2.07 (IQR -3.72 to 0.15) to a median of -1.17 (IQR -2.26 to 0.16) p < 0.05, and 0.24 (IQR -1.09 to 1.84) p < 0.05, respectively, at subsequent angiogram. Nine patients underwent further catheterisation. Four patients underwent complete anatomical repair. Only one patient required unifocalisation, as most patients had a native supply to all-important lung segments. CONCLUSION: Right ventricular outflow tract stenting is a useful procedure in the subset of patients with Tetralogy of Fallot with pulmonary stenosis and major aortopulmonary collateral arteries, where native pulmonary arterial growth is required to facilitate repair.

Diagnosing pulmonary arteriovenous malformations in the presence of atrial septal defect and anomalous pulmonary venous drainage-An imaging challenge.

Saline contrast echocardiography is a simple and effective method to diagnose the presence and type of right-to-left shunt in patients with unexplained cyanosis. It is considered a very sensitive test to diagnose pulmonary arteriovenous malformations. Our patient presented with unexplained cyanosis and transthoracic echocardiography showed an atrial septal defect and anomalous pulmonary venous drainage of the right and left upper pulmonary veins to the superior venacava. We describe how we used saline contrast echocardiography to demonstrate the presence of pulmonary arteriovenous malformations even in the presence of atrial septal defect and anomalous pulmonary venous drainage.

Anomalous systemic and pulmonary veins-An unusual coexistence.

A 12-year-old child presented with recurrent respiratory infections and was diagnosed with Scimitar syndrome. Drainage of IVC and course of aberrant arterial supply from aorta were not clear by echocardiogram, and hence, additional imaging was planned. CT unraveled the presence of a rare combination of anomalous pulmonary venous connection (APVC) to IVC, with near-atresia/severe stenosis of IVC, superior to the drainage of scimitar vein. There was a prominent azygos vein with preferential contrast opacification on lower limb injection.

Haemodynamic consequences following closure of an Abernethy malformation in a patient following a total cavopulmonary shunt.

A 17-year-old girl with situs ambiguous, hypoplastic right ventricle with a large ventricular septal defect, and severe pulmonary stenosis had undergone Kawashima operation 10 years back. She had significant desaturation because of a large Abernethy malformation, with reverse shunting from the inferior caval vein to the portal vein. It was closed with a vascular plug, with improvement in oxygen saturation. She developed extensive inferior caval vein thrombus following the procedure, which was managed conservatively by anti-coagulation.

Imaging the elusive anomalous origin of left coronary artery from pulmonary artery.

A 7-year-old girl with severe mitral regurgitation and pulmonary artery hypertension with good ventricular function was planned for mitral valve repair, but was subsequently diagnosed to have anomalous left coronary artery from pulmonary artery. We describe the pitfalls in the diagnosis of this rare anomaly, particularly in the presence of severe pulmonary artery hypertension and absence of circumstantial evidence of coronary steal and ventricular dysfunction.

Unexpected complication of uncovered aortic end in ductal stenting.

A newborn with tricuspid atresia and pulmonary atresia underwent ductal stenting. The aortic end of the ductus was not completely covered and was wide open; the baby was discharged on dual antiplatelets. The baby presented after a month with desaturation, and an angiogram showed extensive thrombus in the stent and the right pulmonary artery. The angle formed by the stent with the uncovered aortic end is likely to have precipitated the thrombus.

Large Esophageal Hematoma Following Transesophageal Echocardiography-Guided Device Closure of Atrial Septal Defect.

A 47-year-old woman with a large ostium secundum atrial septal defect (ASD) and severe pulmonary artery hypertension underwent device closure of ASD under transesophageal echocardiography guidance. She developed a massive esophageal hematoma which was diagnosed 4 days after the procedure. The use of dual antiplatelets after the device closure further aggravated the hematoma. As the patient remained stable and the site of leak could not be identified by contrast studies, she was managed conservatively with nil per mouth, broad-spectrum antibiotics, and continuous nasogastric aspiration. We were faced with the risk of thromboembolism after stopping antiplatelets versus the risk of increasing peri-esophageal hematoma if they were continued. With careful monitoring for thrombus formation on the device, the antiplatelets were stopped and the hematoma resolved. The hematoma resolved by 10 days, and the antiplatelets were restarted gradually. Iatrogenic esophageal injury is an important cause of esophageal perforation, which is a condition with high mortality and morbidity. Esophageal perforation following device closure of ASD is particularly challenging as the scenario is worsened by the use of antiplatelets and they have to be discontinued with the attendant risk of thromboembolism.

Markedly dilated right atrium in an infant: what is the diagnosis?

An 18-month-old boy presented with recurrent episodes of irritability and documented fast heart rate suggestive of supraventricular tachycardia. Cardiovascular examination revealed significant cardiomegaly, normal heart sounds and no murmurs. The differential diagnosis of marked right atrial dilatation and management principles of idiopathic dilatation of the right atrium are described.

Tetralogy of Fallot with aortic stenosis and common arterial trunk: Is there a morphological overlap?

Tetralogy of Fallot with aortic stenosis is an extremely rare entity which bears some morphological similarities with the common arterial trunk. Through two illustrative cases of TOF with aortic stenosis we describe the shared anatomical peculiarities of the two anomalies with a review of the possible genetic and developmental factors responsible for the association.

Normal pulmonary venous drainage in the setting of total anomalous pulmonary venous connection.

Total anomalous pulmonary venous connection (TAPVC) and anomalous pulmonary venous drainage are not synonymous. This has been described in the setting of right isomerism (bilateral right sidedness) where the pulmonary veins are connected anomalously but drain normally to the left-sided morphological right atrium. We describe another situation in right isomerism where normal pulmonary venous drainage is present in the setting of TAPVC.

Outcomes of neonatal critical congenital heart disease: results of a prospective registry-based study from South India.

OBJECTIVES: Congenital heart disease (CHD) is now a leading contributor of infant and neonatal mortality in many low/middle-income countries including India. We established a prospective neonatal heart disease registry in Kerala to understand presentation of CHD, proportion of newborns with critical defects who receive timely intervention, outcomes at 1 month, predictors of mortality and barriers to timely management. METHODS: The congenital heart disease registry for newborns (≤28 days) in Kerala (CHRONIK) was a prospective hospital-based registry involving 47 hospitals from 1 June 2018 to 31 May 2019. All CHDs, except small shunts with a high likelihood of spontaneous closure, were included. Data on demographics, complete diagnosis, details of antenatal and postnatal screening, mode of transport and distance travelled and need for surgical or percutaneous interventions and survival were collected. RESULTS: Of the 1474 neonates with CHD identified, 418 (27%) had critical CHD, 22% of whom died at 1 month. Median age at diagnosis of critical CHD was 1 (0-22) day. Pulse oximeter screening identified 72% of critical CHD and 14% were diagnosed prenatally. Only 8% of neonates with duct-dependent lesions were transported on prostaglandin. Preoperative mortality accounted for 86% all deaths. On multivariable analysis, only birth weight (OR 2.7; 95% CI 2.1 to 6.5; p<0.0005) and duct-dependent systemic circulation (OR 6.43; 95% CI 5 to 21.8, p<0.0005) were predictive of mortality. CONCLUSIONS: While systematic screening, especially pulse oximetry screening, enabled early identification and prompt management of a significant proportion of neonates with critical CHD, important health system challenges like low use of prostaglandin need to be overcome to minimise preoperative mortality.