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.

Critical decision of operability in congenital heart disease patient with severe pulmonary hypertension.

Repair of congenital heart disease in the presence of high pulmonary pressure has always been a contentious issue. Pulmonary vascular resistance (PVR) is considered important for establishing operability in these patients. However, PVR estimation is not always accurate and cannot solely be relied upon to make critical decision of operability. Clinical examination, chest X‑ray, and echocardiography are also important indicators of pulmonary vascular disease. Knowledge of pits and falls of each investigation is important for appropriate management in these patients. We present a case report of successfully operated, 6‑year‑old child with anomalous origin of the right pulmonary artery from aorta, deemed inoperable on the basis of PVR estimation.

Compression of undiagnosed aberrant right subclavian artery during transesophageal echocardiography probe insertion.

Transesophageal echocardiography (TEE) has become an important monitoring tool for the anesthesiologist during repair of intracardiac defects. Although the incidence of reported complications associated with its use is low, one should be careful during the insertion and use of TEE probe, as it may result in potential devastating problems. We present a case of undiagnosed aberrant right subclavian artery (ARSA) that got compressed by the TEE probe during its insertion. It was noticed because of the presence of the right radial artery catheter, else it would have passed unnoticed.

GastrointentinaI bleed with clopidogrel and aspirin.

Anti-platelet drugs have been used to prevent thrombosis of systemic to pulmonary artery shunts. Aspirin has traditionally been used. Clopidogrel is being studied as an alternative and in combination with aspirin for shunt patients. We report a near fatal gastro-intestinal bleed in a patient with shunt and on aspirin and clopidogrel. This combination has been known to produce similar bleeds. The authors recommend caution in combining them. Prospective studies currently underway should evaluate this aspect of the antiplatelet drugs.

Post-op systemic hypertensive crises in an infant with total anomalous pulmonary venous connection.

Systemic hypertension is not usually a complication following repair of total anomalous pulmonary venous connection (TAPVC). We report an infant with supracardiac TAPVC with hypertensive crises post-operatively resulting in pulmonary edema. We feel this might have been related to the pre-operative hemodynamics as described. Beta-blockers improved the hypertensive crises.

Voriconazole in newborns.

Voriconazole is a newer systemic antifungal agent effective against Candida and Aspergillus. There are few reports of its safe use in newborns. We report the first case series of safe Voriconazole use in critically ill newborns with cardiac disease along with several other cardiac drugs without any significant drug interaction or side-effect.

Innominate artery compression of trachea.

Innominate artery may cross the trachea and cause airway obstruction is a rare cause of vascular obstruction of airway. We describe a child with stridor, inability to extubate in whom the diagnosis was suspected on fluoroscopy and confirmed by angiography. Reimplantation of the innominate artery resulted in excellent results with longterm follow up. Variantions of this condition and modalities for confirmation of diagnosis are discussed.

Central line retrieval in a neonate.

Peripherally inserted central lines are important for management of newborns in intensive care. 1% of PICC lines can migrate. Here we describe a preterm baby with a migrated PICC line enmeshed on the pulmonary valve. This was retrieved by snare from the pulmonary valve. The technique is described as also the challenges related to doing this in a small baby.

Surgical treatment of congenital mitral stenosis: medium-term results

OBJETIVO: Analisar a evolução de pacientes portadores de estenose mitral congênita (EmiC) submetidos a tratamento cirúrgico, buscando estudar os fatores desfavoráveis da plastia valvar e os seus marcadores de durabilidade. MÉTODO: De 1989 a 2002, 23 pacientes foram submetidos a tratamento cirúrgico da EmiC, excluindo aqueles com defeito do septo atrioventricular, e formas univentriculares. A idade mediana foi de 15,5 meses (variação de 2 a 204), e o peso mediano foi de 11 kg (variação 4,5 a 51,6 kg). Anomalias associadas estiveram presentes em 17 (73,9 por cento), complexo de Shone em nove (39,1 por cento) e hipertensão pulmonar em 14 (60,9 por cento). A estenose mitral era grave em 14 (60,9 por cento) pacientes, e moderada no restante (gradiente transmitral mediano de 16 mmHg, variação 8,5 a 32). A plastia valvar foi realizada em 18 (78,3 por cento) pacientes e a troca em cinco (21,7 por cento). As técnicas de plastia incluiram a papilarotomia (n=10), ressecção de membrana supravalvar (n=9) e comissurotomia (n=8). Doze (52,2 por cento) pacientes requereram procedimentos associados. RESULTADOS: Não houve óbitos precoces ou tardios num seguimento médio foi de 58,5 ñ 46,7 meses (variação 1 a 156 meses). O tempo médio de hospitalização foi de 12,7 ñ 8,2 dias. Não foram encontrados fatores de risco associados a insucesso da plastia valvar. A curva atuarial livre de reoperaçäes em cinco anos foi de 67,1 por cento (IC 95 por cento: 56,8 por cento a 77,4 por cento). O grupo submetido a plastia valvar necessitou de reoperação em oito (44,4 por cento) pacientes, sendo dois precoces e seis tardios, comparados com uma (20 por cento) reoperação no grupo submetido à troca valvar. A presença de hipertensão pulmonar no pré-operatório esteve relacionada significativamente (p<0,005) a maiores taxas de reoperação. Todos os pacientes, exceto dois, encontram-se em classe funcional I da NYHA e o ecocardiograma mostra no máximo insuficiência e/ou estenose mitral leve. CONCLUSåES: As reoperaçäes foram o maior fator de morbidade no seguimento a médio prazo e a hipertensão pulmonar pôde ser considerada o fator adverso de durabilidade da plastia mitral, a qual foi o tratamento de escolha para a estenose mitral congênita.