Upper airway obstruction after pediatric cardiac surgery is not uncommon. In the cardiac surgical population, an important etiology is vocal cord paresis or paralysis following extubation. In this study, we aimed to evaluate the feasibility and accuracy of ultrasonography (US) assessment of the vocal cords mobility and compare it to fiber-optic laryngoscope (FL). A prospective pilot study has been conducted in Pediatric Cardiac ICU (PCICU) at King Abdulaziz Cardiac Center (KACC) from the 1st of June 2009 till the end of July 2010. Patients who had cardiac surgery manifested with significant signs of upper airway obstruction were included. Each procedure was performed by different operators who were blinded to each other report. Results of invasive (FL) and non-invasive ultrasonography (US) investigations were compared. Ten patients developed persistent significant upper airway obstruction after cardiac surgery were included in the study. Their mean ± SEM of weight and age were 4.6 ± 0.4 kg and 126.4 ± 51.4 days, respectively. All patients were referred to bedside US screening for vocal cord mobility. The results of US were compared subsequently with FL findings. Results were identical in nine (90%) patients and partially different in one (10%). Six patients showed abnormal glottal movement while the other four patients demonstrated normal vocal cords mobility by FL. Sensitivity of US was 100% and specificity of 80%. US assessment of vocal cord is simple, non-invasive and reliable tool to assess vocal cords mobility in the critical care settings. This screening tool requires skills that can be easily obtained.

Arrhythmias after pediatric cardiac surgery are common and can be life-threatening. They occur intraoperatively or may appear shortly after surgery during postoperative care. They require early management and specific intervention. In this review, we describe important critical arrhythmias that are encountered during postoperative management of children undergoing cardiac surgery. We review the diagnosis, management, and explain the role of epicardial electrocardiogram in diagnosing certain types of postoperative rhythm abnormalities seen during early period after pediatric cardiac surgery.

Chylothorax is the accumulation of chyle in the pleural cavity, which usually develops after disruption of the thoracic duct along its intra-thoracic route. In the majority of cases, this rupture is secondary to trauma (including cardio thoracic surgeries). Chylothorax is a potentially serious complication after cardiovascular surgeries that require early diagnosis and adequate management. This study aims to determine the risk factors and the impact of chylothorax on the early postoperative course after pediatric cardiac surgery. A retrospective study of all cases complicated with chylothorax after pediatric cardiac surgery was conducted at King Abdulaziz Cardiac Center between January 2007 and December 2009. There were 1135 cases operated on during the study period. Of these, 57 cases (5%) were complicated by chylothorax in the postoperative period. Thirty patients (54%) were males, while 27 (47%) were females. Ages ranged from 4 to 2759 days. The most common surgeries complicated by chylothorax were the single ventricle repair surgeries (15 cases, 27%); arch repairs (10 cases, 18%); ventricular septal defect repairs (10 cases, 18%); atrioventricular septal defect repairs (7 cases, 12%); arterial switch repair (6 cases, 11%), and others (8 cases, 14%). The intensive care unit (ICU) and the length of hospital stays were significantly longer in the chylothorax group. Additionally, some early postoperative parameters such as incidence of sepsis, ventilation time, and inotropes duration and number were higher in the chylothorax group. Chylothorax after pediatric cardiac surgery is not a rare complication. It occurs more commonly with single ventricle repair and aortic arch repair surgeries, and has a significant impact on the postoperative course and post operative morbidity.

Congenital heart defects (CHDs) are the most common types of birth defects, and global incidence of CHDs is on the rise. Despite the prevalence of CHDs, the genetic determinants of the defects are still in the process of being identified. Herein, we report a consanguineous Saudi family with three CHD affected daughters. We used whole exome sequencing (WES) to investigate the genetic cause of CHDs in the affected daughters. We found that all affected individuals were homozygous for a novel splice-altering variant (NM_001330069.1: c.265-1G>T) of PRKD1, which encodes a calcium/calmodulin-dependent protein kinase in the heart. The homozygous variant was found in the affected patients with Pulmonary Stenosis (PS), Truncus Arteriosis (TA), and Atrial Septal Defect (ASD). Based on the family’s pedigree, the variant acts in an autosomal recessive manner, which makes it the second autosomal recessive variant of PRKD1 to be identified with a link to CHDs, while all other previously described variants act dominantly. Interestingly, the father of the affected daughters was also homozygous for the variant, though he was asymptomatic of CHDs himself. Since both of his sisters had CHDs as well, this raises the possibility that the novel PRKD1 variant may undergo autosomal recessive inheritance mode with gender limitation. This finding confirms that CHD can be associated with both dominant and recessive mutations of the PRKD1 gene, and it provides a new insight to genotype–phenotype association between PRKD1 and CHDs. To our knowledge, this is the first report of this specific PRKD1 mutation associated with CHDs.