Chitra Narasimhan

Treatment of congenital non-ductal shunt lesions with the amplatzer duct occluder II.

Amplatzer Duct Occluder II (ADO II) is especially designed for closing long ducts in infants. The experience with off‐label use of ADO II in non‐ductal positions is limited. Aim: To evaluate feasibility of use of ADO II in non‐ductal positions, incidence of complete heart block (CHB), advantages and disadvantages. Material and Results: Out of 79 defects closed with ADO II, 61 had perimembranous, 12 apical/mid‐muscular ventricular septal defects (VSD), 4 Gerbode defects, one each of coronary arterio‐venous fistula and aorto‐right ventricular tunnel. Age ranged from 8 months to 21 years (mean 8.9 ± 4.02 years, median 9 years). The mean fluoroscopy time was 5.2 ± 1.1 min with range 4.2–9.2 min. Complete closure was achieved in all. Three cases developed transient junctional bradycardia, treated with steroids. One patient who developed CHB (1.3%) needed temporary pacing. Discussion: ADO II is a low profile device. It can be easily delivered through a 5F guiding catheter and needs very short fluoroscopic time as arteriovenous loop is not needed. The cost is 1/3 of regular ventricular septal occluders. The CHB that is a major challenge for closure of VSDs is less common with soft, specially designed ADO II, which does not compress the conducting system. Conclusions: Use of the ADO II in non‐ductal positions can be achieved with high success and low complication rates, especially CHB; its use is also associated with significantly reduced procedure time and device cost. Device size availability restricts use of the ADO II to defects up to 6 mm in diameter.

Purulent Pericarditis with Quadruple Valve Endocarditis

Patient: Male, 7 Final Diagnosis: Purulent pericarditis with quadruple valve endocarditis Symptoms: — Medication: (4S,4aS,5aR,12aS)-9-[2-(tert-butylamino)acetamido]-4,7 bis(dimethylamino)-1,4,4a,5,5a,6,11,12aoctahydro-3,10,12,12a-tetrahydroxy-1,11-dioxo-2 naphthacenecarboxamide Clinical Procedure: Pericardiocentisis Specialty: Cardiology Objective: Rare disease Background: Infective endocarditis (IE) is a disease with a highly varied clinical picture. Spread of the infection to the pericardium from the infective endocardium is uncommon and IE involving all 4 cardiac valves is also a very rare occurrence, being more common in intravenous drug users (IVDU). Case Report: A 7-year-old boy had purulent pericarditis with infective endocarditis (IE) on all 4 cardiac valves and vegetation in the left ventricular and right atrial cavity. Culture of the pericardial fluid grew methicillin-resistant staphylococcus aureus (MRSA) sensitive to tigecycline. The child made a dramatic improvement with tigecycline treatment. Conclusions: Aggressive management with pericardiocentesis and appropriate antibiotics can show remarkable clinical improvement. Tigecycline can be used safely and effectively as a life-saving drug in children.

Renal sympathetic denervation for treatment of resistant hypertension – Indigenous technique

BACKGROUND The endovascular approach of ablation of renal sympathetic nerves is found to be effective in the treatment of uncontrolled hypertension. We report here our experience with the procedure in eight patients with drug resistant hypertension. METHODS We included patients in whom the blood pressure remained above 150/90 mmHg despite being on minimum three antihypertensive drugs. Radiofrequency ablation of the sympathetic nerves of both the renal arteries was done using conventional ablation catheters. The patients were followed at 1-month, 3 months and 6 months post procedure and blood pressure recorded. RESULTS All patients underwent successful renal sympathetic denervation. The mean blood pressure of the patients was 181/102.5 mmHg before the procedure and the average requirement of antihypertensive drugs per day was 4. A significant reduction in both systolic and diastolic blood pressure was observed post procedure which sustained over the follow up period of six months. The mean blood pressure observed at 1-month, 3 and 6 months were 137.5/80 mmHg, 136/81 mmHg and 137.5/81 mmHg, respectively. The average requirement of the number of antihypertensives also was reduced to 2.5 at the end of 6 months. There were no procedural complications. CONCLUSION Catheter based renal denervation causes substantial and sustained blood pressure reduction without serious adverse events in patients with resistant hypertension.

A rare anomaly: ‘hemitruncus’

A 7-year-old boy with a history of frequent respiratory tract infections in infancy, presented with easy fatigability and exertional dyspnoea. On examination, he had mild cyanosis, clubbing, left parasternal heave and a loud pulmonary component of the second heart sound with no murmurs. An electrocardiogram showed right ventricular hypertrophy with …

Unusual cases of right-sided and left-sided May-Thurner syndrome.

May-Thurner syndrome is a rare clinical entity involving venous obstruction of the left lower extremity. The May-Thurner syndrome is a phenomenon commonly described as an acquired stenosis of the left common iliac vein secondary to compression of the left common iliac vein between the right common iliac artery and the underlying vertebral body. We report one case of May-Thurner syndrome, and another rare case of reverse May-Thurner syndrome, incidently detected during intervention, in a case of aortic stenosis and mitral stenosis with dextrocardia and situs inversus.

Percutaneous Device Closure of Patent Ductus Arteriosus with Pulmonary Artery Hypertension: Long-Term Results

UNLABELLED Device closure of patent ductus arteriosus (PDA) is treatment of choice. But device closure in presence of pulmonary artery hypertension (PAH) remains a challenge. Data on patient selection, technical considerations, and complications are limited. AIM To know the challenges and efficacy of device closure of PDA with PAH. MATERIALS AND RESULTS Out of 1,325 cases of device closure of PDA, 246 (18.6%) with PAH formed the study material. To test the feasibility, chosen device is used to occlude PDA for ten minutes without oxygen inhalation. The device is released only if PAH reduced. PAH decreased in all except in 1 patient after closure with muscular ventricular septal occluder (MVSDO), pulmonary artery pressure (PAP) transiently increased (became supra-systemic), without significant reduction in aortic pressure. Device embolized in 8 patients (3.3%). Percutaneous retrieval was done in 4 (by snare in 2 and by fixing the cable to device in 2) and replaced with bigger devices. The surgical removal of the embolized MVSDO and ligation was done in 4 cases. All patients were on oral sildenafil and bosentan until PAP regressed to normal. Follow up was from 6 months to 9 years. No residual shunt in any patient on follow-up. The PAP regressed to normal in all except 5 cases (2.03%) of Downs syndrome with systemic PAP. CONCLUSIONS Device closure of PDA with PAH is feasible, safe in all age groups. Temporary PDA occlusion with device is effective and time saving for evaluating pulmonary vascular reactivity. Device embolization in aorta is higher with severe PAH. Novel method of retrieval is effective.

Functionally significant, novel GATA4 variants are frequently associated with Tetralogy of Fallot

Transcription factor GATA4 is known to play crucial role during heart development, regulating expression of several other key cardiogenic factors. Various GATA4 mutations are reported in familial as well as sporadic cases of congenital heart defects (CHDs). To estimate the prevalence and pathogenic potential of GATA4 variants in our CHD cohort, we have screened 285 CHD cases along with 200 controls by Sanger sequencing and identified 9 genetic variants (c.23C>A; p.Ala8Asp, c.25G>A; p.Ala9Thr, c.223G>T; p.Ala75Ser, c.383A>T; p.Glu128Val, c.397A>T; p.Ser133Cys, c.682T>A; p.Trp228Arg, c.1064C>G; p.Thr355Ser, c.1073G>C; p.Ser358Thr, and c.1220C>A; p.Pro407Gln) in 22 unrelated CHD probands (frequency:7.72%). Five of these are novel and located in the N‐terminal transactivation domain (TAD) and first zinc finger domain. Majority C‐terminal domain variants are polymorphic. Two of the TAD variants p.Glu128Val, p.Ser133Cys, and a first zinc finger variant p.Trp228Arg, impair combinatorial synergy of NKX2‐5, SRF, and TBX5, suggesting potential role of these domains in GATA4 interactions with these factors. Decreased DNA‐binding affinity with EMSA also supports this observation. Homology modeling and tertiary structure comparison show conformational changes in these variants. Interestingly, GATA4 variants are more frequently associated with ToF (45%; P = 0.0046) and PS (22.7%; P < 0.0001) in spite of abundance of septal defects in our study cohort.

Transposition of great arteries with natural partial Senning: A rare case report

The association of transposition of the great arteries (TGA) and anomalous pulmonary venous connection is extremely rare. Children with transposition of the great arteries improved dramatically with the advent of the atrial repair. In this report, we describe a 40-day old male infant with TGA and associated anomalous pulmonary venous connection who presented with the history of cyanosis and hurried breathing. This patient underwent successful balloon atrial septostomy and discharged with uneventful recovery.

Percutaneous closure of pseudoaneurysm of common iliac artery with amplatzer duct occluder II

Seven-year-old boy presented with pain in right lower limb and abdomen after a fall from a bullock cart. He was referred for management of pseudoaneurysm of the right common iliac artery. After CT angiogram, the vascular surgeon opined that child was not suitable for surgical patch or graft or endovascular stenting as there was no landing zone for the stent. Hence the child was treated with transcatheter closure with two Amplatzer duct occluder II (ADO II). On follow-up the symptoms and bruit disappeared. To the best of our knowledge this is the first case of pseudoaneurysm, treated with ADO II.