Vasoactive-ventilation-renal score and outcomes in infants and children after cardiac surgery.

Introduction: There is a need to index important clinical characteristics in pediatric cardiac surgery that can be obtained early in the postoperative period and accurately predict postoperative outcomes. Methodology: A prospective cohort study was conducted in the pediatric cardiac ICU and ward on all children aged <18 years undergoing cardiac surgery for congenital heart disease from September 2018 to October 2020. The vasoactive-ventilation-renal (VVR) score was analyzed to predict outcomes of cardiac surgeries with a comparison of postoperative variables. Results: A total of 199 children underwent cardiac surgery during the study period. The median (interquartile range) age was 2 (0.8-5) years, and the median weight was 9.3 (6-16) kg. The most common diagnoses were ventricular septal defect (46.2%) and tetralogy of Fallot (37.2%). At the 48th h, area under the curve (AUC) (95% CI) values were higher for the VVR score than those for other clinical scores measured. Similarly, at the 48th h, AUC (95% CI) values were higher for the VVR score than those for the other clinical scores measured for the length of stay and mechanical ventilation. Discussion: The VVR score at 48 h postoperation was found to best correlate with prolonged pediatric intensive care unit (PICU) stay, length of hospitalization, and ventilation duration, with the greatest AUC-receiver operating characteristic (0.715, 0.723, and 0.843, respectively). The 48-h VVR score correlates well with prolonged ICU, hospital stay, and ventilation.

Renal Outcomes in Neonates and Infants with Transposition Physiology Undergoing Arterial Switch Procedure.

Acute kidney injury (AKI) in children with Transposition of Great arteries (TGA) undergoing Arterial Switch operation (ASO) is an important complication in the post-operative period associated with worse outcomes. AKI in children post open cardiac surgery has been well studied, with lesser data in literature pertaining to TGA and its sub-types specifically. This was a prospective, observational study enrolling infants with TGA undergoing ASO at a single center over a span of a decade from January 2010 to December 2020. The infants were followed during the duration of ICU and hospital stay, with documentation of baseline and intraoperative parameters as well as post-operative course. Out of 145 infants enrolled in the study, 83.1% developed AKI with majority (83.9%) having stage 1 AKI. Higher odds of AKI were seen in infants requiring Norepinephrine [odds ratio - 16.76 (95% CI 2.19-128.2), p < 0.001] and those who developed gram-negative infections [2.81 (1.04-7.56), p - 0.036]. Infants with AKI had significantly higher vasoactive-inotropic support at day 1 than those without AKI [16 (12.5-21.50 vs 13 (10.25-15.75), p - 0.014]. Seventeen infants in the AKI group (14%) died as opposed to none in the non-AKI group (p = 0.076). Median hours of ventilator support required were significantly higher in those with AKI than those who did not develop AKI (48 vs 45.5 p = 0.015). The infants with ASO + ASD + PDA (53% of neonates who died) were younger, had less weight at admission, more gram-negative sepsis and need for dopamine, as compared to ASO + VSD + ASD (23.5% of mortality) and ASO + ASD + VSD + aortic arch repair (23.5% of mortality). AKI in infants with TGA undergoing ASO is common and associated with poorer outcomes. In this subpopulation, AKI development is associated most commonly with hemodynamic instability and infections. This is the first study, looking at outcomes of TGA depending on the sub-types of ASO surgeries done in the infants [ASO with ASD + PDA or ASD + VSD or ASD + VSD + Arch Repair].

Hypocalcemic cardiomyopathy-different mechanisms in adult and pediatric cases.

BACKGROUND: Hypocalcemic cardiomyopathy (CMP) is a rare but potentially reversible cause of heart failure. However, the mechanism of hypocalcemia seems to differ between infants and adults. Although severe vitamin D deficiency alone is the usual cause of hypocalcemic CMP in infants, in adult patients significant cardiac dysfunction usually occurs as a result of hypoparathyroidism, either isolated or in combination with vitamin D deficiency. We present two cases of hypocalcemic CMP-one adult and one pediatric-to highlight these differences. CASE PRESENTATION: The first patient was a 47-year-old female who presented with progressive dyspnea and fatigue and was found to have severe left ventricular (LV) systolic dysfunction (LV ejection fraction, 25%). Her serum calcium level was 3.5 mg/dL, serum phosphorus level was 5.7 mg/dL, and serum 25-hydroxyvitamin D level was 14.1 ng/mL, along with a serum PTH level of 11.8 pg/mL. Her LV ejection fraction normalized completely over 6 months with calcium and calcitriol treatment. In contrast, the second patient was an infant who had presented in cardiogenic shock. Investigations revealed serum calcium of 4.5 mg/dL, serum phosphorus of 11.9 mg/dL, 25-hydroxyvitamin D of 8.9 ng/mL, and serum PTH level of 670 pg/mL. Calcium and calcitriol supplementation resulted in rapid and complete clinical and hemodynamic recovery. CONCLUSION: Hypocalcemia is a rare but treatable cause of dilated CMP. In infants, hypocalcemia is usually due to maternal vitamin D deficiency and is accompanied by compensatory hyperparathyroidism. In contrast, in adult patients, hypocalcemic CMP is usually a result of hypoparathyroidism, with or without concomitant vitamin D deficiency.

A case of Kartagener's syndrome: Importance of early diagnosis and treatment.

Kartagener's syndrome is a very rare congenital malformation comprising of a classic triad of sinusitis, situs inversus and bronchiectasis. Primary ciliary dyskinesia is a genetic disorder with manifestations present from early life and this distinguishes it from acquired mucociliary disorders. Approximately one half of patients with primary ciliary dyskinesia have situs inversus and, thus are having Kartagener syndrome. We present a case of 12 year old boy with sinusitis, situs inversus and bronchiectasis. The correct diagnosis of this rare congenital autosomal recessive disorder in early life is important in the overall prognosis of the syndrome, as many of the complications can be prevented if timely management is instituted, as was done in this in this case.

A case of Kartagener's syndrome: Importance of early diagnosis and treatment.

Kartagener's syndrome is a very rare congenital malformation comprising of a classic triad of sinusitis, situs inversus and bronchiectasis. Primary ciliary dyskinesia is a genetic disorder with manifestations present from early life and this distinguishes it from acquired mucociliary disorders. Approximately one half of patients with primary ciliary dyskinesia have situs inversus and, thus are having Kartagener syndrome. We present a case of 12 year old boy with sinusitis, situs inversus and bronchiectasis. The correct diagnosis of this rare congenital autosomal recessive disorder in early life is important in the overall prognosis of the syndrome, as many of the complications can be prevented if timely management is instituted, as was done in this in this case.

Is it really PPHN? Think before starting sildenafil!

The use of sildenafil has become a common practice in neonatal intensive care unit on clinical ground, because opinion by Pediatric Cardiologist is usually not available especially in peripheral centers. We consider it essential to share our experience that severe pulmonary arterial hypertension can be due to some unusual hemodynamics or extremely rare structural causes which do not require pulmonary vasodilator therapy.

Assessment of operability in d-transposition of great arteries with ventricular septal defect: A practical method.

INTRODUCTION: Pulmonary vascular disease is a risk factor in the surgical management of patients with d-transposition of great arteries (d-TGA) and a ventricular septal defect (VSD). In older infants or children with this physiology, the question of operability often arises. Cardiac catheterization in this condition can be fallacious. It is well known that oxygen reduces pulmonary arterial pressure and pulmonary vascular resistance especially where irreversible pulmonary vascular obstructive disease has still not set in. We tried to implement this effect of oxygen in correlation with echocardiography in patients with TGA-VSD physiology where operability was in question. METHODS: Patients with d-TGA and a large post tricuspid shunt in whom operability was considered doubtful were selected for the study. We administered humidified oxygen at the rate of 10 litres/minute by mask for 48 hours in the ward or intensive care unit. After administration of oxygen we reassessed the child echocardiographically looking for signs of lowering of pulmonary vascular resistance which included increased pulmonary venous blood flow to the left atrium (LA) and right to left shunting across the VSD. OBSERVATION: We studied 1 patient with d-TGA and aortopulmonary window (APW), 4 patients with TGA / large VSD and 1 patient with Taussig-Bing anomaly. The age of the studied children ranged from 4 months to 3 years with a mean age of 1.1 years. After administering oxygen as described, echocardiogram showed an increase in pulmonary venous blood flow to the LA and right to left shunting across the VSD in 5 patients and increased flow reversal in aorta in presence of the APW. CONCLUSION: Patients with TGA/VSD physiology with doubtful operability can be subjected to this method of determining operability using echocardiography after administering oxygen. Although not 100% accurate in predicting long term postoperative pulmonary hypertension, this is a simple, noninvasive method that can aid in decision making in such a situation.

Effect of task related circuit training on walking ability in a Multiple Sclerosis subject. A single case study.

OBJECTIVE: To investigate the effectiveness of task related circuit training on walking ability in a Multiple Sclerosis subject. DESIGN: Single case study of a man diagnosed with moderate primary progressive type of multiple sclerosis. METHOD: Baseline measurement of lower limb muscle strength, speed test, Timed "Up and Go" test and 6-minute walk test, Modified fatigue impact scale and Expanded Disability Status Score were taken. After baseline measurement, subject was explained the sequence of tasks to be used in circuit training and the subject was given task related circuit training for 12 weeks. Post training measurements for all the outcome measures were taken. SETTING: Department of Occupational Therapy, NIOH, Kolkata, West Bengal, India. PARTICIPANT: A 34 -year -old male. INTERVENTION: Task related Circuit training for a session of 45 minutes, 3 days per week for 12 weeks. RESULTS: The subject showed improvement in speed test, step test, 6-minute Walk Test. Also, fatigue was reduced. The walking ability of the subject improved, with increase in muscle strength, endurance, and physical fitness. CONCLUSION: Task-related circuit training is effective in improving muscle strength and endurance, and in decreasing the fatigue of the subject thereby improving the subject's ability to walk.