Anuradha Sridhar

Hybrid intraoperative pulmonary artery stenting in redo congenital cardiac surgeries

OBJECTIVE Reconstruction of branch pulmonary arteries (PAs) can be challenging in redo congenital heart surgeries. Treatment options like percutaneous stent implantation and surgical patch angioplasty may yield suboptimal results. We present our experience with hybrid intraoperative stenting which may be an effective alternative option. METHODS We retrospectively analyzed data of all patients with PA stenosis who underwent intraoperative PA branch stenting in our institution between January 2011 and December 2012. RESULTS Ten patients [6 females, median age 10 (1.4 to 37) years], underwent hybrid stenting of the PA. Primary cardiac diagnoses were pulmonary atresia with ventricular septal defect (VSD) in three patients, pulmonary atresia with intact ventricular septum in two, Tetralogy of Fallot (TOF) in one, Double outlet right ventricle (DORV) with pulmonary stenosis (PS) in one, complex single ventricle in two and VSD with bilateral branch PA stenosis in one patient. Concomitant surgeries were revision/reconstruction of RV-PA conduit in 4, Fontan completion in 4, repair of TOF with conduit placement in 1 and VSD closure in 1 patient. The left PA was stented in 7, the right in 2 and both in 1, with a total of 11 stents. There were no complications related to stent implantation. Two early postoperative deaths were unrelated to stent implantation. At mean follow-up period of 14.8 (12-26) months, stent position and patency were satisfactory in all survivors. None of them needed repeat dilatation or surgical reintervention. CONCLUSION Hybrid stenting of branch PA is a safe and effective option for PA reconstruction in redo cardiac surgeries. With meticulous planning, it can be safely performed without fluoroscopy.

Giant Coronary and Systemic Aneurysms of Kawasaki Disease in an Infant

Kawasaki disease (KD) was diagnosed in a 4-month-old male infant after 4 days of fever, rash, and bulbar conjunctivitis. He received two doses of intravenous immunoglobulin (IVIG), high-dose oral acetylsalicylic acid, and three doses of methylprednisolone within in the first week of illness for persistent fever. During the third week of his illness, echocardiogram showed giant aneurysms at the origin of the right coronary artery (17 mm) and at the bifurcation of the left coronary artery (9 mm). Anticoagulant therapy was started. At 9-month follow-up, the child was clinically well, and there were no ST-T wave changes on electrocardiogram. Aortography showed a giant aneurysm (19 9 19 mm) at the origin of the right coronary artery and another aneurysm (9 9 17 mm) distally (Figs. 1 and 2). The left coronary artery showed a large aneurysm (10 9 6 mm) at the bifurcation (Fig. 1). There was no evidence of stenosis in the right or left coronary artery. Subclavian artery injections showed large aneurysms in both the right (8 9 12 mm) and left (12 9 24 mm) brachial arteries (Fig. 3). Abdominal and carotid angiography were normal. At present, the infant is being maintained on low-dose aspirin and anticoagulant therapy. The international normalized ratio (INR) has been maintained between 2 and 2.5 (Fig. 4). KD is an acute multisystem vasculitis of unknown etiology that predominantly affects smalland medium-sized arteries. Fifteen percent of KD occurs in patients from 6 months to 4 years of age and often presents as the incomplete form [1]. Giant aneurysms (diameter [ 5 mm) are considered a high risk factor for the long-term development of stenotic coronary lesions [2]. Administration of IVIG within 10 days of illness decreases the incidence of giant aneurysms [3]. The risk of coronary artery aneurysms is higher for patients who do not respond to the first dose of IVIG, as happened in our case [3]. Systemic artery aneurysms have been reported to occur in 2% of patients with KD, and they are often associated with coronary aneurysms [4]. Fig. 1 Aortography showing a large aneurysm at the origin of the right coronary artery (white arrow) and a large aneurysm at the bifurcation of the left main coronary artery (red arrow). (Color figure online)

Anatomically corrected malposition of great arteries

Anatomically corrected malposition of great arteries (ACMGA) is a rare form of congenital heart disease in which the great arteries arise above the anatomically correct ventricles but have abnormal spatial relationship. We report the case of a 26-year-old female with ACMGA and tunnel type of subaortic obstruction. The abnormal relationship and segmental arrangement necessitates systematic approach in evaluation for proper diagnosis and surgical repair. This unusual case is reported for its rarity and to highlight the need for awareness to differentiate it from other more common conditions.

Cardiac surgery-associated acute kidney injury in a developing country: Prevalence, risk factors and outcome.

Little is known about cardiac surgery-associated acute kidney injury (CS-AKI) in children in developing regions of the world. The study aimed to determine the prevalence of CSAKI, associated factors and its impact on mortality and utilization of hospital services. The hospital records of children aged 0-17 years who underwent CS at an Indian hospital were reviewed. CS-AKI was defined as a rise in serum creatinine of ≥0.3 mg/dL in any 48 h and or by urine output <0.5 mL/kg/h for an 8-h period in the first five days after CS. The study included 323 children with a median age of one year (0.04-17), of whom 22 (6.8%) were neonates and 18.3% had a single ventricle. About 60% of the children had Risk Adjusted Congenital Heart Surgery-I category 1 or 2 interventions. CS-AKI occurred in 39 children (12.1%). Factors associated with CS-AKI were sepsis and intraand post-operative hypotension. In-hospital mortality was six-fold higher in children who developed CS-AKI. CS-AKI was associated with two to three days more of mechanical ventilation and Intensive care unit stay. CS-AKI occurs in children in developing countries, but at a lower frequency mainly due to the predominance of post-neonatal children undergoing less-complex CSs. CS-AKI was associated with higher in-hospital mortality and increased utilization of hospital services. Factors associated with CS-AKI included intraand post-operative hypotension and sepsis.

Arterial Tortuosity Syndrome

As its name suggests, arterial tortuosity syndrome is characterized by blood vessel abnormalities, particularly abnormal twists and turns (tortuosity) of the blood vessels that carry blood from the heart to the rest of the body (the arteries). Tortuosity arises from abnormal elongation of the arteries; since the end points of the arteries are fixed, the extra length twists and curves. Other blood vessel abnormalities that may occur in this disorder include constriction (stenosis) and abnormal bulging (aneurysm) of vessels, as well as small clusters of enlarged blood vessels just under the skin (telangiectasia).

Cruciate Fenestration in Ventricular Septal Defect Patch for High-Risk Patients With High Pulmonary Vascular Resistance

Late presentation of patients with large ventricular septal defect (VSD) and elevated pulmonary vascular resistance (PVR) is not uncommon in developing countries. Surgical VSD closure in these patients carries risks of persistent pulmonary hypertension, right ventricular failure, and mortality. Several techniques for creation of valved patches or fenestrated patches have been developed to address these issues. We have successfully used a simple and easily reproducible technique in which a cruciate fenestration is created in the patch used for VSD closure.

Do preoperative haemodynamic data and reactivity test predict the postoperative reversibility of pulmonary arterial hypertension in patients with large ventricular septal defect and borderline operability

Background Decisions to operate on patients with shunt lesions presenting late with severe pulmonary arterial hypertension (PAH) and borderline operability are often not based on precise cut off values of haemodynamic data owing to paucity of studies. Objective To assess the reliability of the preoperative haemodynamic data and reactivity test in predicting the postoperative reversibility of PAH in patients with isolated large ventricular septal defects (VSDs) and borderline operability. Patients and method Between 2004 and 2010, 30 patients underwent VSD closure surgically; no early deaths occurred. Twenty-six patients were followed up regularly (mean 39.6±16 months) and one late postoperative death occurred. Fourteen patients who had been followed up for at least 1 year postoperatively underwent cardiac catheterisation. Results There were 3 responders (asymptomatic patients with pulmonary vascular resistance (PVR) index <3 WU.m2) and 12 non-responders. The following were lower among responders: mean age at surgery (3.2±0.42 vs 11.55±3.29 years, p=0.227), mean baseline PVR index (3.69±0.8 vs 10.57±9.1, p=0.204), average resistance ratio (RR=0.25±0.01 vs 0.59±0.25, p=0.049) and ratio of pulmonary and systemic mean pressures (PAm:SAm ratio) (0.70±0.009 vs 0.87±0.118, p=0.003). Conclusions Preoperative ‘base line’ PAm:SAm and RR appear to be better predictors of postoperative outcome than other baseline parameters. Preoperative reactivity test had no significant role in predicting postoperative reversibility of PAH at mid-term.

Unusual Combination of Hypoplastic Left Ventricle, Atrioventricular Septal Defect With Restrictive Ventricular Septal Defect, and Common Arterial Trunk:

We describe rare cases of common arterial trunk (truncus arteriosus communis) with unbalanced atrioventricular septal defect, left ventricular hypoplasia, and restrictive ventricular septal defect. The embryology, hemodynamics, and the clinical implications of this complex combination are discussed.

The curious case of a button which led to the needle.

Foreign bodies in the heart are uncommon in children. These are often removed even if asymptomatic to prevent complications like erosion, embolization, bleeding, thrombosis, and endocarditis. We report the case of a one-and-a-half-year-old child with a hypodermic needle in the heart which was found incidentally and removed successfully by surgery.

Mycotic Aneurysm Complicating a Covered Stent Implanted for Coarctation of the Aorta in a Child

A mycotic aneurysm associated with a covered stent in the thoracic aorta of a 12-year-old child was successfully managed by excision and replacement with aortic homograft. On follow-up, there was unobstructed flow through the homograft. This case highlights the need for high index of suspicion for mycotic aneurysm and prompt surgical intervention in children with coarctation of aorta who present with features of infective endocarditis.