Congenital Complete Heart Block Complicated by Atrial Flutter Diagnosis and Management.

Seventeen-month-old child was diagnosed in utero to have congenital complete heart block. The mother has Sjogren's syndrome with high Anti Ro antibodies. The baby was delivered at term with a heart rate of 55-60 beats per minute. Echocardiography revealed a structurally normal heart with a small atrial septal defect and moderate patent ductus arteriosus. At the age of 17 months, he developed atrial flutter which was aborted using electrical cardioversion in the Cath lab. No recurrence of the atrial flutter during a one-year follow-up.

Assessing Pediatric Inter-Hospital Transfer: A single-center, Retrospective, Observational Study of Saudi Arabia's National Life-Saving Protocol.

Objective: To examine the accuracy of our national Life-Saving Protocol (LSP). To the best of our knowledge, this is the first study addressing this issue in Saudi Arabia. Background: LSP was created to facilitate triaging patients with LIFE or LIMB threatening conditions in peripheral hospitals with limited services to large regional hospitals to receive definitive care. Method: This is a retrospective single-center observational study over 12 months studying the patients who arrived via LSP to our Emergency room (ED), at the only regional pediatric hospital. For the subgroup of patients who were admitted to PICU through LSP, we further assessed their outcomes like mortality and length of stay (LOS) through a matched case-control study of 1:1 with similar patients who were admitted to our PICU via other routes rather than LSP. The primary outcome is to assess the accuracy of the LSP in triaging pediatric patients with LIFE of LIMB conditions. Secondary outcomes include assessing the association between LSP and (mortality, LOS) for those who were admitted to the regional PICU via LSP compared to patients admitted to PICU via other sources of admission. Results: During the study period, 118 patients arrived at our ED via LSP. Only 43 patients (36 %) were admitted to the PICU with LIFE or LIMB conditions. A total of 64 patients (54%) of the patients were admitted directly to the general pediatric ward from ED level due to absence of LIFE of LIMB threatening condition and 8% (n=9) were discharged immediately home from the ED level due to lack of any significant illness. One patient died at ED level, and one was referred to another hospital with a minor orthopedic injury. For those who were admitted to the PICU via LSP, the mortality rate was (13.9%) (6/43), and the control group was (4.6%) (2/43) with a p-value of 0.08. Conclusion: LSP is an excellent initiative and essential tool in our healthcare system; however, our study showed huge variation in the ability of the system to recognize true pediatric patients with LIFE or LIMB conditions. Our study might form a stepping-stone in future studies assessing the LSP at the national level.

Pulmonary hypertension associated with congenital heart disease; clinical decision scenario.

Pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) is one type under group 1 PH. Undiagnosed or delayed diagnosis of significant CHD might lead to significant PAH and at the end might lead to Eisenmenger syndrome. We could expect the degree of PAH in patients with CHD by proper clinical assessment as well as by the basic assessment tools including the chest x-ray (CXR), ECG, and transthoracic echocardiography (TTE). We are presenting a three and half years old child with a delayed/missed diagnosis of large patent ductus arteries (PDA) who present with significant PAH. Clinical evaluation, CXR, ECG, TTE, as well as cardiac catheterization data are presented, with a review of the current guidelines regarding the management of pediatric patients with PAH-CHD.

Cardiac Catheterisation Interventions in Neonates and Infants Less Than Three Months.

INTRODUCTION: Pediatric cardiac catheterization interventions become an established way of care for selected patients with congenital heart diseases. Cardiac catheterization for neonates and small infants can be challenging. The indications for diagnostic cardiac catheterization have decreased with the advent of advanced non-invasive imaging modalities. PATIENTS AND METHOD: Between June 2012 and July 2017 patients less than three months who had cardiac catheterization in two centers were reviewed. RESULTS: During the study period, 174 patients underwent interventional cardiac catheterization,83.3% of them had CHD with two-ventricle circulation and 29 patients (16.7%) had single ventricle pathophysiology. Procedures include diagnostic cath, BAS, balloon pulmonary and aortic valvuloplasty, coarctation angioplasty, and stenting procedures. The vascular access depends upon the type of procedure. All except one had general anesthesia. ICU admission was required on 106 patients (62%). Patients were divided according to the type of cardiac lesion (single versus biventricular pathology) as well as according to the type of intervention (stenting and non-stenting procedures). Comparing these groups revealed that: stent procedures and procedures for patients with single ventricle pathologies were performed at an earlier age, with more contrast, fluoro and procedure time than for non-stent procedures and procedures for patients with biventricular pathologies. Complications include transient arrhythmias in most patients, perforation of the RVOT in one and lower limb hypoperfusion in 12 patients. ICU complications include low cardiac output symptoms (LCOS) in 10 (7%), and sepsis in 8. No intra-procedure mortality. The overall survival was 94%. Ten patients died, with one early and 9 late mortality. 60% of the dead patients had PDA stenting. Reintervention varies according to the patient's diagnosis. CONCLUSION: Cardiac catheterization intervention an important modality in the management of neonates and infants with critical CHD. Well planned procedures and team expertise are essential. Stenting procedures and procedures for patients with single ventricles carries higher morbidity and mortality.

Lethal recurrent mycotic ascending aortic pseudoaneurysm in a 21-month-old child with repaired subaortic membrane.

Mycotic pseudoaneurysm of the aorta is a rare and lethal complication of pediatric congenital heart surgery. We report the lethal consequences of recurrent mycotic pseudoaneurysm in an 18-month-old baby, early after subaortic membrane resection. We managed to repair the pseudoaneurysm successfully by replacing the infected ascending aorta using bovine jugular vein graft, but unfortunately, the patient developed new pseudoaneurysm at the site of anastomosis which led to his death. Although prompt diagnosis and surgical management can save the patient life, uncontrolled infection can lead to the recurrence of the problem and lethal results.

Percutaneous removal of air-bullet gunshot: case report and literature review.

BACKGROUND: Cardiac air bullet injuries are rare but can be associated with significant morbidity and mortality. CASE PRESENTATION: We are presenting a young male child who sustained an accidental injury to the chest by an air rifle. Bullet entered the right ventricle from the anterior part of the chest and was identified in the RV side of the interventricular septum by echocardiography and chest CT scan. There was mild pericardial effusion but no valvular injury. The bullet was removed in the cath lab, and the patient was discharged home on the second day. CONCLUSIONS: It is reasonable to try foreign body removal in the cath lab, for certain cases, and avoid cardiac surgery.

Aortopulmonary window with anomalous right coronary artery from the pulmonary artery. Case report and literature review.

Aortopulmonary window is a rare congenital heart lesion. It might be associated with other CHDs, as well as with anomalous origin of the coronary arteries. Anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is the most commonly described coronary artery anomaly in association with aortopulmonary window. We are describing a premature neonate who was diagnosed to have aortopulmonary window and ARCAPA immediately after birth, and had a successful operation at the age of 4 months. This report highlights the importance of very careful assessment of the coronary arteries in patients with aortopulmonary window.

Treatment options for transposition of the great arteries with ventricular septal defect complicated by pulmonary vascular obstructive disease.

The arterial switch operation is the procedure of choice for patients with isolated transposition of the great arteries or those with associated atrial and/or ventricular septal defects. After the development of pulmonary arterial hypertension, the surgical options for patients with a late presentation include either retraining the left ventricle by pulmonary artery banding followed by an arterial switch operation or palliative atrial or arterial switch, with or without medical management of pulmonary hypertension. We present a case with D-transposition of the great arteries with ventricular septal defects and irreversible pulmonary arterial hypertension who improved after a palliative atrial switch operation.