Inhaled iloprost as an add-on therapy for advanced pulmonary arterial hypertension: An Indian perspective

BACKGROUND Pulmonary arterial hypertension (PAH) is a progressive disease with high morbidity and mortality. Risk stratification and initiation of dual or triple combination therapy has a better clinical response, especially in high-risk patients. Unfortunately, prostacyclin analogues are not marketed in India; hence, the use of these medications is limited. We report the benefits and difficulties of using iloprost inhalation in patients with advanced PAH in India. METHODS In this prospective observational study, we included patients with group 1 PAH. Inhaled iloprost was initiated as an add-on therapy for patients who had clinical, echocardiographic or laboratory deterioration on dual oral medications. Patients with clinical instability were excluded. All patients underwent thorough clinical evaluation, detailed echocardiogram and laboratory investigations. Patients were started on inhaled iloprost 2.5 ?g six times daily and closely followed up. The dose was escalated if necessary. On follow-up, clinical echocardiographic and laboratory evaluation was done on all patients. RESULTS Fourteen patients (11 women) with a median age of 32 years (2–66 years) with group 1 PAH were started on inhaled iloprost as an add-on therapy. Improvement in clinical parameters, WHO functional class, echocardiographic-derived right ventricular function, and N-terminal pro-brain natriuretic peptide (NT-pro-BNP) levels were observed in 10 of 14 patients. A median increase of 31% (4.2, 106%) in the distance travelled during 6-minute walk test, a median increase of 45% (–20, 120%) in right ventricular fractional area change, a median increase of 27% (–16.7, 60%) in tricuspid annular peak systolic excursion and a median decrease of 36.7% (–69.6, 17.2%) in NT-pro-BNP levels were observed after initiation of medication. Three patients had progression of symptoms and were then referred for lung/heart–lung transplant. One patient developed progression of symptoms after an excellent initial response and transitioned to subcutaneous treprostinil. Improvement in clinical, echocardiographic and laboratory features allowed us to successfully perform surgical Potts shunt in 2 patients. The medications were well tolerated with minimal and transient side-effects. There were no deaths. CONCLUSION Inhaled iloprost can be used with acceptable benefits and minimal side-effects in patients with PAH.

Indian Guidelines for Indications and Timing of Intervention for Common Congenital Heart Diseases: Revised and Updated Consensus Statement of the Working Group on Management of Congenital Heart Diseases. Abridged Secondary Publication

ustification: A number of guidelines are available for management of congenital heart diseases from infancy to adult life. However,these guidelines are for patients living in high income countries. Separate guidelines, applicable to Indian children, are required whenrecommending an intervention for congenital heart diseases, as often these patients present late in the course of the disease and mayhave co-existing morbidities and malnutrition. Process: Guidelines emerged following expert deliberations at the National ConsensusMeeting on Management of Congenital Heart Diseases in India, held on 10th and 11th of August 2018 at the All India Institute of MedicalSciences, New Delhi. The meeting was supported by Children’s HeartLink, a non-governmental organization based in Minnesota, USA.Objectives: To frame evidence based guidelines for (i) indications and optimal timing of intervention in common congenital heartdiseases; (ii) follow-up protocols for patients who have undergone cardiac surgery/catheter interventions for congenital heart diseases.Recommendations: Evidence based recommendations are provided for indications and timing of intervention in common congenitalheart diseases, including left-to-right shunts (atrial septal defect, ventricular septal defect, atrioventricular septal defect, patent ductusarteriosus and others), obstructive lesions (pulmonary stenosis, aortic stenosis and coarctation of aorta) and cyanotic congenital heartdiseases (tetralogy of Fallot, transposition of great arteries, univentricular hearts, total anomalous pulmonary venous connection, Ebsteinanomaly and others). In addition, protocols for follow-up of post surgical patients are also described, disease wise.

Effect of dexmeditomidine on postoperative junctional ectopic tachycardia after complete surgical repair of tetralogy of Fallot: A prospective randomized controlled study.

Introduction: Incidence of junctional ectopic tachycardia (JET) after repair of tetralogy of Fallot (TOF) is 5.6–14%. Dexmeditomidine is a α-2 adrenoceptor agonist modulates the release of catecholamine, resulting in bradycardia and hypotension. These effects are being explored as a therapeutic option for the prevention of perioperative tachyarrhythmia. We undertook this study to examine possible preventive effects of dexmedetomidine on postoperative JET and its impact on the duration of ventilation time and length of Intensive Care Unit stay. Methods: After obtaining approval from the hospitals ethics committee and written informed consent from parents, this quasi-randomized trial was initiated. Of 94 patients, 47 patients received dexmedetomidine (dexmedetomidine group) and 47 patients did not receive the drug (control group). Results: Dexmedetomidine group had more number of complex variants like TOF with an absent pulmonary valve or pulmonary atresia (P = 0.041). Hematocrit on cardiopulmonary bypass (CPB), heart rate while coming off from CPB and inotrope score was significantly low in the dexmedetomidine group compared to control group. The incidence of JET was significantly low in dexmedetomidine group (P = 0.040) compared to control group. Conclusions: Dexmedetomidine may have a potential benefit of preventing perioperative JET.

Single therapeutic catheterization for treatment of native coarctation of aorta and large patent ductus arteriosus using a covered stent.

A 20-year-old girl was diagnosed to have severe coarctation of the aorta, large patent ductus arteriosus and severe pulmonary artery hypertension. She underwent single therapeutic catheterization for the treatment of native coarctation of aorta and closure of ductus arteriosus using 39 mm long Cheatham-Platinum covered stent. The procedure was done successfully under local anesthesia without any complication. This appears to be a safe strategy while dealing with an adolescent or an adult with this combination of lesions.

Stenting stenosed aortopulmonary collateral arteries in pulmonary atresia with ventricular septal defect.

We present two cases of pulmonary atresia with ventricular septal defect who were not suitable for corrective surgery due to absent or hypoplastic native pulmonary arteries and were quite symptomatic following shunt surgery. We dilated and stented stenosed aortopulmonary collaterals as palliative procedure with improvement in oxygen saturation, and significant symptomatic relief.

Temporary endoepicardial atrioventricular sequential pacing for complete heart block following complex surgery for congenital heart disease.

Complete heart block following intracardiac surgical repair for complex congenital heart disease is not uncommon. In the presence of ventricular dysfunction, ventricular pacing alone may not improve the cardiac output. We report the feasibility and efficacy of endoepicardial atrioventricular sequential pacing in a case of postoperative complete heart block.

Extrinsic obstruction of the main coronary arteries in a child with hypertrophic cardiomyopathy.

Phasic narrowing of the coronary arteries on angiography is a well-known entity in both children and adults and has been described in relation to all epicardial arteries. There is a high incidence of myocardial bridges in hypertrophic cardiomyopathy. We report the case of a 6-year-old girl with hypertrophic obstructive cardiomyopathy who had extrinsic obstruction of the left main and right coronary arteries.