High take off left main coronary artery accompanied by multicryptic left ventricle myocardium detected by cardiac computerized tomography in a young male: case report

Background Myocardial crypts are discrete, narrow, blood filled invaginations within the left ventricular myocardium and high-take-off coronary artery are rare manifestations where coronary arteries originate above the sinotubuler junction. Case presentation A 41-year-old man with multiple coronary artery disease risk factors admitted to our outpatient department with progressive dyspnea and atypical chest pain. Physical examination revealed no pathological findings. His blood examination revealed only mild to moderately high IgE and LDL levels. Transthoracic echocardiography (TTE) was normal. His treadmill test was normal, yet in the 3rd stage of the test he had an atypically located chest pain which was relieved in the resting period. As he had multiple cardiovascular risk factors, we performed a coronary CT angiography to exclude coronary artery disease. Coronary CT angiography(CCTA) demonstrated multiple myocardial crypts, a muscular VSD like defect which were not detectable with TTE and a high take off left main coronary artery (LMCA). After CCTA, we repeated the TTE to investigate the crypts and VSD-like defect which were clear on CCTA, yet a precise TTE hardly showed crypts and didn’t confirm a shunt between the left and right ventricle. We defined the defect as ‘spontaneously closed muscular VSD’. None of these pathologies were clinically relevant with the patient’s symptoms, thus pneumonology started a montelukast therapy for 1 year and we decided to follow up the patient, as multiple crypts may indicate an early phase hypertrophic cardiomyopathy. Conclusions Considering that a high take-off LMCA is a congenital anomaly, encountering multiple crypts which are also congenital pathologies, is plausible, as congenital anomalies may accompany eachother. Echocardiography is a very useful, practical imaging tool but regrettably may be suboptimal due to various patient and method related reasons. Target combination of different cardiovascular imaging tools like echocardiography, cardiac CT(CCT), may be utilized in order to ensure a comprehensive diagnosis particularly.

Transcatheter Closure of a Large Muscular Ventricular Septal Defect (VSD) in a Child Weighing Five Kilogram with Severe Pulmonary Arterial Hypertension

Ventricular septal defect (VSD) is the commonest congenial heart lesion which may close spontaneously in significant number of cases. Muscular VSD has better chance of spontaneous closure. Some of the large muscular VSDs may cause severe complications like pulmonary hypertension and intractable heart failure. Closing VSD in young infant is challenging specially with devices as delivery systems are too large comparing to size of femoral vessels. Some centre prefers hybrid procedure of per ventricular device closure as surgical closure is high risk in young infants with complications. Here a case of large muscular VSD with severe pulmonary hypertension is reported in a ten month old girl which was closed successfully with a Konar- MFTM device and patient was discharged after 24 hours. JAFMC Bangladesh. Vol 16, No 2 (December) 2020: 84-86

Radiation dose typical values per procedure complexity for transcatheter ventricular septal defect closure in pediatrics

This study aims to establish radiation dose typical values (TV) for ventricular septal defect (VSD) percutaneous closures based on patient-, device- and procedure-related characteristics. Clinical and exposure data were retrospectively reviewed for 92 consecutive pediatric patients with VSD in whom transcatheter closure attempt was undertaken between March 2016 and December 2019 at a single reference center. TV were derived as the median value of total air kerma-area product (PKA,T), air kerma at the patient entrance reference point, fluoroscopy time and number of frames. Statistical analyses were conducted to investigate the exposure variability according to patient, device- and procedure-related characteristics. For (5–< 15 kg) patients, TV were 27 Gy · cm2 in term of PKA,T for venous vs. 9 Gy · cm2 for arterial approach. For (15–< 30 kg) patients, TV were 53 Gy · cm2 for muscular vs. 18 Gy · cm2 for perimembranous defects and 36 Gy · cm2 for AmplatzerTM vs. 14 Gy · cm2 for the LifetechTM device. Procedures conducted with the assistance of a fellow, venous approach, muscular VSD, AmplatzerTM devices, and procedural incidents increased the exposure levels significantly. The established TV provide benchmarks on patient exposure per procedure complexity and should help promote better patient radiation protection.

CATHETER BASED CLOSURE OF HYPERTENSIVE PDA(S) WITH UNCONVENTIONAL DEVICES –IMMEDIATE AND SHORT TERM FOLLOW UP

Patent ductus arteriosus (PDA) is a common form of CHD, accounts for approximately 8% of congenital heart disease with the incidence of one in 2500 to 5000 live births. More common in female. It may be asymptomatic and is sometimes not diagnosed early resulting into prolonged abnormal aorto-pulmonary shunt which may result in silently progressing hypertension and left ventricular dysfunction. This study aims to evaluate the immediate &short-term follow up results after TCC of large PDAs with severe pulmonary arterial hypertension (PHT) in children.This is a observational study of 38 patients with Large PDA and severe PHT who were referred to this centre for treatment during the period from July 2015 to Feb 2018 . After evaluating clinical and hemodynamic parameters reversibility of severe PAH was established and transcatheter closure of PDA was performed. Patients were followed up clinically and echocardiographically at 24 hours, 1 month, 3 months, 6 months, 12 months and 24 months to assess the efficacy, safety of the procedure & its impact on PHT. Thirty eight patients, (26 females, 12males) patients between 6 months to 16 years of age had underwent successful device closure. 18 patients had PDA sizes between 6-8 mm,12 had between 8- 10 mm , 6 had between 10-15mm and only 2 patients had largest PDA more than 15 mm at PA end . 5 patients were selected for closure with muscular VSD device . We have successfully closed 3 of our largest PDA (12 mm , 16mm and 18mm) with Post Infarct Muscular VSD Occluder measuring 20 mm,22 mm and 24 mm respectively. TCC of hypertensive but reversible PDA is feasible. The immediate and short-term outcomes have proven this method to be safe and valid.

A hybrid procedure for the closure of a large muscular ventricular septal defect in a 6-month-old infant

Background: Transthoracic device closure (TTDC), also known as a Hybrid procedure, has been proposed as an alternative, less invasive approach compared to open-heart surgery for the treatment of ventricular septal defect (VSD). Case Presentation: We present our first national case of TTDC in a 6-month-old female baby with a muscular 8mm ventricular septal defect, 3 mm atrial defect, enlarged right and left ventricle and a dilated pulmonary artery complicated by severe pulmonary hypertension.Treatment consisted of two pulmonary artery banding attempts at the age of 2 months to control pulmonary hypertension – the interventions were combined with diuretics and angiotensin-converting enzymes inhibitors. Yet, the initial approach was suboptimal as we noticed a failure to thrive continuous sweating and tachypnea. Because of the worsening condition at the age of 6 months, and a weight of 6.6 kg, we performed TTDC. After median sternotomy, a 10mm muscular VSD occluder was implanted under trans-oesophagal echocardiography guidance on the beating heart. The procedure lasted 90 min and was performed without incident; the hemodynamics were stable with only a minor residual VSD. The child was extubated after 2 hours and discharged after five days from the hospital.Conclusions: Transthoracic device closure (TTDC) is a promising treatment modality for large muscular VSD in small infants with low weight. TTDC is feasible in cases with heavy myocardial right ventricle trabeculae and who previously underwent open-heart surgery.

A hybrid procedure for the closure of a large muscular ventricular septal defect in a 6-month-old infant

Background: Transthoracic device closure (TTDC), also known as a Hybrid procedure, has been proposed as an alternative, less invasive approach compared to open-heart surgery for the treatment of ventricular septal defect (VSD). Case Presentation: We present our first national case of TTDC in a 6-month-old female baby with a muscular 8mm ventricular septal defect, 3 mm atrial defect, enlarged right and left ventricle and a dilated pulmonary artery complicated by severe pulmonary hypertension.Treatment consisted of two pulmonary artery banding attempts at the age of 2 months to control pulmonary hypertension – the interventions were combined with diuretics and angiotensin-converting enzymes inhibitors. Yet, the initial approach was suboptimal as we noticed a failure to thrive continuous sweating and tachypnea. Because of the worsening condition at the age of 6 months, and a weight of 6.6 kg, we performed TTDC. After median sternotomy, a 10mm muscular VSD occluder was implanted under trans-oesophagal echocardiography guidance on the beating heart. The procedure lasted 90 min and was performed without incident; the hemodynamics were stable with only a minor residual VSD. The child was extubated after 2 hours and discharged after five days from the hospital.Conclusions: Transthoracic device closure (TTDC) is a promising treatment modality for large muscular VSD in small infants with low weight. TTDC is feasible in cases with heavy myocardial right ventricle trabeculae and who previously underwent open-heart surgery.

A hybrid procedure for the closure of a large muscular ventricular septal defect in a 6-month-old infant

Background: Transthoracic device closure (TTDC), also known as a Hybrid procedure, has been proposed as an alternative, less invasive approach compared to open-heart surgery for the treatment of ventricular septal defect (VSD). Case Presentation: We present our first national case of TTDC in a 6-month-old female baby with a muscular 8mm ventricular septal defect, 3 mm atrial defect, enlarged right and left ventricle and a dilated pulmonary artery complicated by severe pulmonary hypertension.Treatment consisted of two pulmonary artery banding attempts at the age of 2 months to control pulmonary hypertension – the interventions were combined with diuretics and angiotensin-converting enzymes inhibitors. Yet, the initial approach was suboptimal as we noticed a failure to thrive continuous sweating and tachypnea. Because of the worsening condition at the age of 6 months, and a weight of 6.6 kg, we performed TTDC. After median sternotomy, a 10mm muscular VSD occluder was implanted under trans-oesophagal echocardiography guidance on the beating heart. The procedure lasted 90 min and was performed without incident; the hemodynamics were stable with only a minor residual VSD. The child was extubated after 2 hours and discharged after five days from the hospital.Conclusions: Transthoracic device closure (TTDC) is a promising treatment modality for large muscular VSD in small infants with low weight. TTDC is feasible in cases with heavy myocardial right ventricle trabeculae and who previously underwent open-heart surgery.