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.

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.

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.

Clinical profile and outcomes of childhood dilated cardiomyopathy - A single-center three-decade experience.

Introduction and Aims: Dilated cardiomyopathy (DCM) is an important cause of heart failure (HF) among children. Research on pediatric DCM remains surprisingly scarce. The primary objective of the study was to evaluate the clinical profile and outcomes of pediatric DCM and the secondary objective was to study the predictors of outcome. Methods and Results: We enrolled all patients with cardiomyopathy who presented to us between 1990 and 2020 and were younger than 18 years. During the 30-year study period, we identified 233 cases of pediatric cardiomyopathy. One hundred and nineteen (51%) cases had DCM. This retrospective cohort was analyzed to study their outcome and the possible predictors of outcome. Nearly, 8% presented in the neonatal period, and 37% in infancy. The most common mode of presentation was dyspnea on exertion (71%). Ninety-three patients presented in heart failure (78%). The median left ventricular dimension z-score in diastole was 4.3 (range 2.5-9.06). The median left ventricle (LV) ejection fraction was 31%. Seventy-two percent of this cohort were on angiotensin-converting-enzyme inhibitors, 40% on aldosterone antagonists, and 47% on beta-blockers. One-third had syndromic, metabolic, genetic, or any secondary cause identified. Twenty-seven patients satisfied the three-tiered clinical classification for the diagnosis of probable acute myocarditis. Over a mean follow-up of 3.29 years, 27% were lost to follow-up. Among the remaining patients who were on follow-up (n = 86), 39 (45%) died, 31 (36%) recovered, and 16 (18%) had persistent LV dysfunction. Heart Failure was the most common cause of death. Eight patients in this cohort (4.2%) had thromboembolic phenomena. Nine had sustained ventricular arrhythmias and six had atrial/junctional arrhythmias. Among the various risk factors studied, only infantile onset had a significant relationship with death or ventricular arrhythmias (P value- 0.05). The 5-year survival rate of DCM patients was 59%. Conclusion: A reasonably good percentage of our population showed recovery of the left ventricular function (36%). Only infantile onset had a significant relationship with death or ventricular arrhythmias. The outcome in our DCM cohort is similar to other population cohorts.

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.

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.

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.

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.

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.

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.

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.

Influence of age at surgery on left ventricular strain in patients with anomalous origin of left coronary artery from pulmonary artery.

OBJECTIVES: Myocardial strain abnormalities are described after surgical repair of anomalous left coronary artery from pulmonary artery (ALCAPA) even after recovery of ventricular function. The factors that predispose to the presence of these strain abnormalities in the presence of normal ventricular function are unknown. The aim of this study was to find out whether the age at repair influences the presence of global and regional strain abnormalities on follow-up. METHODS: Repaired ALCAPA patients from a single centre (n = 40) with good ventricular ejection fraction on follow-up were recruited. Baseline and follow-up data were collected from electronic records. Global and regional myocardial strain assessment was done by speckle tracking echocardiography prospectively. The association between age at repair and strain abnormalities on follow-up was analysed. RESULTS: The patients who presented earlier had significantly worse ventricular function pre-operatively compared to older patients (P < 0.0005). Global longitudinal strain was abnormal in 40% of patients with normal ventricular ejection fraction on follow-up. Presence of longitudinal strain abnormalities was more in patients who underwent repair at older age than in those who were repaired earlier (P < 0.0005). The probability of having normal longitudinal strain on follow-up was 81.6% if surgery was done before 7.8 months of age. If operated before 6 months, the odds of having normal myocardial strain was 11 times higher. Regional strain abnormalities of varying severity were present in all patients in the left and in some patients in the right coronary artery territories. CONCLUSIONS: Older age at ALCAPA repair is associated with increased incidence of myocardial strain abnormalities. Regional strain abnormalities were found in both left and right coronary artery territories.

Dual left anterior descending artery with anomalous left anterior descending artery from pulmonary artery: double trouble.

Anomalous origin of left anterior descending artery (LAD) from pulmonary artery (ALADCAPA) with dual LAD from left sinus is an extremely rare coronary anomaly. The diagnostic challenge lies in differentiating this from a coronary cameral fistula. Surgical reimplantation of the anomalous LAD is recommended to prevent the risk of myocardial ischaemia and ventricular arrhythmias.

Major Aortopulmonary Collateral Arteries.

Major aortopulmonary collateral arteries (MAPCAs) are congenital vessels that arise from the aorta or its first-order branches and are distally connected to the pulmonary arterial vasculature, thereby providing pulmonary blood flow. MAPCAs are commonly associated with several congenital heart diseases that have compromised pulmonary circulation due to severe stenosis involving pulmonary valves or arteries or due to pulmonary atresia. Embryologically, MAPCAs are presumed to be persistent segmental arteries. MAPCAs can be imaged with CT and MRI, and such imaging findings are important for surgeons and interventionists. The management options for MAPCAs include unifocalization, surgical ligation, and endovascular interventions, such as coil embolization. This review highlights the role of reporting certain critical features of MAPCAs at CT and MRI, which will help to facilitate management decisions for systemic-to-pulmonary collateral vessels observed in patients with congenital heart disease. Keywords: Pediatrics, CT Angiography, Image Postprocessing, Interventional-Vascular, MR Angiography, Embolization, Stents, Cardiac, Vascular, Aorta © RSNA, 2022.

Repercussions of a giant major aortopulmonary collateral aneurysm adjacent to vertebral artery origin.

An aneurysm of major aortopulmonary collateral in an adult with congenital cyanotic heart disease was detected incidentally following a hemoptysis episode. The location and size of the aneurysm needed special concern during treatment to avoid aneurysm-related complications and thromboembolism secondary to intervention.

Pulmonary artery aneurysm: Harbinger of an ominous disease.

An 11-year-old girl presented with chronic cough and recurrent hemoptysis. On examination, she had features of right heart failure and cyanosis, with severe pulmonary hypertension on echocardiogram. Computed tomography pulmonary angiography showed aneurysmal dilatations of the pulmonary artery with elevated erythrocyte sedimentation rate and C-reactive protein and positive human leukocyte antigen B51. A diagnosis of Hughes-Stovin syndrome (vascular variant of Behcet's syndrome) was confirmed, and she was started on immunosuppression, on which there was improvement.

Thrombosis of aneurysmal pulmonary arteries in patent ductus arteriosus with Eisenmenger syndrome.

A 45-year-old lady with patent ductus arteriosus with Eisenmenger's syndrome had presented with hemoptysis. Computed tomography revealed aneurysmally dilated pulmonary arteries with a large calcified organized thrombus.

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.