Omar Tamimi

Repair of Congenital Heart Disease with Associated Pulmonary Hypertension in Children: What are the Minimal Investigative Procedures? Consensus Statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI)

Standardization of the diagnostic routine for children with congenital heart disease associated with pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignment to repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance) may be detrimental and associated with poor outcomes. Thus, members of the Congenital Heart Disease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct a survey aimed at collecting expert opinion from different institutions in several countries, covering many aspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnostic procedures and immediate postoperative support. In privileged communities, the vast majority of children with congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation, and have an uneventful postoperative course, with no residual PAH. However, a small percentage of patients (older at presentation, with extracardiac syndromes or absence of clinical features of increased pulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk of complications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach, including invasive procedures. The authors emphasize that decision making regarding operability is based not only on cardiac catheterization data but also on the complete diagnostic picture, which includes the clinical history, physical examination, and all aspects of noninvasive evaluation.

Cardiac catheterization in children with pulmonary hypertensive vascular disease: consensus statement from the Pulmonary Vascular Research Institute, Pediatric and Congenital Heart Disease Task Forces

Cardiac catheterization is important in the diagnosis and risk stratification of pulmonary hypertensive vascular disease (PHVD) in children. Acute vasoreactivity testing provides key information about management, prognosis, therapeutic strategies, and efficacy. Data obtained at cardiac catheterization continue to play an important role in determining the surgical options for children with congenital heart disease and clinical evidence of increased pulmonary vascular resistance. The Pediatric and Congenital Heart Disease Task Forces of the Pulmonary Vascular Research Institute met to develop a consensus statement regarding indications for, conduct of, acute vasoreactivity testing with, and pitfalls and risks of cardiac catheterization in children with PHVD. This document contains the essentials of those discussions to provide a rationale for the hemodynamic assessment by cardiac catheterization of children with PHVD.

Successful surgical pulmonary artery reconstruction in arterial tortuosity syndrome.

The outcomes of surgical repair for diffuse pulmonary artery stenoses due to arterial tortuosity syndrome are unknown. We report a 1-year-old girl with tortuosity syndrome who presented with suprasystemic right ventricular pressure due to distortion and stenoses of the pulmonary artery branches. We describe the operative findings of this unique pathology and the surgical techniques used to completely reconstruct the pulmonary arterial tree, with normalization of the right ventricular pressure.

Surgical experience in the rehabilitation and reimplantation of disconnected pulmonary arteries and its effectiveness in restoring pulmonary haemodynamics and function.

OBJECTIVES We established a policy to restore disconnected pulmonary arteries (PAs), regardless of aetiology or cardiac pathology, using a protocol-based strategy. There are conflicting reports of the need to restore disconnected PA especially if severely hypoplastic with some groups advocating surgical repair of cardiac defects to single lung with moderate to high risk. METHODS All patients referred to our PA reconstruction programme (2007-14) with complete separation of one branch PA were included. Our surgical strategy was based on the size of the PA. In patients with mild or no PA hypoplasia, we performed single-stage implantation of the disconnected PA and repair of intracardiac defects. Two-stage approach was used in patients with severe PA hypoplasia where the disconnected PA was retrieved inside the lung then connected to the aorta with a shunt to promote growth. After 6-12 months, the PA was implanted with simultaneous repair of intracardiac defects. RESULTS Twenty patients were included (age range = 1 week to 8 years). Aetiologies were ductal origin of distal PA in 13 patients and iatrogenic in 7 patients. Group 1 (isolated lesion) consisted of 8 patients who presented with severe pulmonary hypertension. Group 2 consisted of 12 patients with associated intracardiac defects and was subdivided into Group 2a (biventricular hearts) 8 patients and Group 2b (univentricular hearts) 4 patients. Single-stage repair was achieved in 10 patients. Direct tissue-to-tissue connection was achieved in 18 (90%) patients. The follow-up was 100% complete (median = 53.8 months). All patients are alive and asymptomatic. Implanted PAs are patent with good growth demonstrated on serial echocardiography. Only two PAs required balloon dilatation for moderate restenosis at the anastomosis. No surgical reintervention was needed. All Group 1 patients had complete resolution of the pulmonary hypertension. Lung perfusion scans showed increased uptake (24-53% of the total uptake). CONCLUSIONS Our study demonstrated that, in paediatric age group, disconnected PAs can be restored with low risk and excellent outcomes even in patients with severely hypoplastic PA. Strategies to repair intracardiac defects to a single lung might not be the optimal approach.

Repair of aortic arch atresia with diffuse hypoplasia of the descending thoracic aorta

We report successful repair of a rare combination of aortic arch atresia and diffuse long-segment hypoplasia of the descending aorta in a 2-month-old infant with PHACES association. Intraoperative findings and surgical techniques are discussed.

Saudi guidelines on the diagnosis and treatment of pulmonary hypertension: 2014 updates

The Saudi Association for Pulmonary Hypertension (previously called Saudi Advisory Group for Pulmonary Hypertension) has published the first Saudi Guidelines on Diagnosis and Treatment of Pulmonary Arterial Hypertension back in 2008.[1] That guideline was very detailed and extensive and reviewed most aspects of pulmonary hypertension (PH). One of the disadvantages of such detailed guidelines is the difficulty that some of the readers who just want to get a quick guidance or looking for a specific piece of information might face. All efforts were made to develop this guideline in an easy-to-read form, making it very handy and helpful to clinicians dealing with PH patients to select the best management strategies for the typical patient suffering from a specific condition. This Guideline was designed to provide recommendations for problems frequently encountered by practicing clinicians involved in management of PH. This publication targets mainly adult and pediatric PH-treating physicians, but can also be used by other physicians interested in PH.

Outcome ventricular septal defect Closure using the Nit-O

Introduction The main goal is to study outcome ventricular septal defect Closure using the Nit-Occlud Le VSD-Coil. Methodology Following IRB approval from KAIMRC with number RC17/285/R, a retrospective chart review analysis were conducted of all pediatric patients with VSD who underwent transcatheter closure of VSD at King Abdul-Aziz Cardiac Center during the period from 2011till 2016. Demographic data, diagnosis, intraprocedure and post-procedure events were reviewed. Results The patients referred for VSD interventional closure are 46 patients, 29 cases fulfill the criteria for VSD closure using Nit-Occlud Le VSD-Coil, twenty-four patients of them had successful interventional closure of VSD with successful rate of 83% and 5 cases were referred to surgery due to device not fit or hemolysis. In this study, age group from 2 years up to 18 years. Ninety percent of VSD is the perimembranous (PM) type, 26 patients, and 3 patients (10%) with muscular VSD. From the 26 PM VSD cases 10 with TV accessory tissue partially covering the VSD defects. Immediate closure of the defect was achieved in 11/24 patients (45%); in five patients, there was tiny residual and 8 with small residual, at 6 months follow up only 6 patients with tiny residual VSD defect. Conclusion The Nit-Occlud”-Le VSD coil device with high safety level used for selected cases of VSDs. The device has minimal effects on the aortic and tricuspid valve, and there was no permanent AV block especially in patients with PM VSDs. Patients with a residual shunt should be followed, especially for the hemolysis.

Different types of isolation of arch branches using cardiac imaging tools

Introduction The goal of this study is to describe (1) different types of isolation of arch branches and (2) to show the importance of careful evaluation of arch morphology using imaging tools such as echocardiography, cardiac computerized tomography (CT) and cardiac catheterization. Methodology Following IRB approval from KAIMRC with number RC17/298/R, we reviewed retrospectively a consecutive case series of pediatric patients who were diagnosed to have isolated innominate or subclavian arteries at King Abdul-Aziz and Prince Sultan Cardiac Centers by use of imaging techniques mentioned above. Data was collected, including demographic information, cardiac diagnosis, diagnostic modalities, and surgical interventions. Results There were a total of 11 patients who were diagnosed to have isolated arch branches. 9 of these patients (82%) had a right aortic arch (RAA) while 3 patients (27%) were found to have a left-sided aortic arch (LAA). Three patients from those with documented RAA had isolated left innominate artery with retrograde blood supply from the circle of Willis through the left vertebral artery and the isolated artery was connected to the pulmonary artery by patent ductus arteriousus or its remnant ligament; the remaining six patients with RAA presented with an isolated left subclavian artery. 3 patients were found to have LAA arch with an isolated left subclavian artery. Of the 11 patients included in this study, one patient had normal intracardiac anatomy, 3 patients a VSD, 2 patients an interrupted aortic arch type B, 2 patients had tetralogy of Fallot, one patient left isomerism, another one hypoplastic left heart syndrome, and the last patient presented with crossed PAs and LPA stenosis. Conclusion Isolation of arch branches is a rare condition, but if it is present a careful initial assessment of arch morphology during echocardiography examination is mandatory; however, the utilization of cardiac CT with expert people reading the results will improve the detecting rate of such a lesion significantly.

Spontaneous resolution of plastic bronchitis in a patient post hemi-Mustard/bidirectional Glenn atrial switch procedure in the double-switch operation for congenitally corrected transposition of great arteries after course of Augmentin

We report the case of a five-year-old girl with plastic bronchitis after repaired complex congenital heart disease, who became asymptomatic after a short course of Augmentin. We report the disease regression as response either to antibiotic or as coincidental with spontaneous resolution.

SHA 085. Heart defects in acyanotic pediatric patients referred with heart murmurs

Video presentation Objectives: Coronary artery bypass graing associated with Ross Procedure performed to 9 years old boy. Dr. Imad Naja; Dr. Nani Najm. Department of Cardiac Sciences, King Abdul-Aziz cardiac Centre, Riyadh, Saudi Arabia. This uncommon presentation of this disease at this age, leads u to share our experience. How to do it is the video presentation of CABG + ROSS performed to a 9 years old boy. Methods: 9 years old boy presented at emergency department with severe chest pain, subsequent Echocardiography showed thickened aortic valve with moderate stenosis. The angiogram showed moderate aortic valve stenosis and severely disease ascending aorta. Result: The surgery was uneventful; it was remarkable the atheromatosis at the level of the ascending aorta, successful outcome, the patient was discharged at the 7th post-operative day. doi:10.1016/j.jsha.2011.02.085 SHA 085. Heart defects in acyanotic pediatric patients referred with heart murmurs Dhafer O. Alqahtani, MD, Ali A. Alakhfash, MD, Omar R Tamimi, MD, Dhafer O. Alqahtani, MD, Ali A. Alakhfash, MD, Omar R Tamimi, MD KACC, Riyadh, Saudi Arabia E-mail address: qahtanidh@ngha.med.sa (D.O. Alqahtani) Objectives: The aim of the study is to explore the prevalence of congenital heart disease in pediatric patients referred to our center solely based on the detection of a heart murmur on routine physical examination. Study design: A retrospective database review was performed for all patients referred to KACC from July 2007 to March 2009 for cardiovascular evaluation because of a heart murmur detected during routine physical examination. This study included all pediatric patients from the neonatal period to 12 years of age who underwent echocardiography in our center. Patients with cyanosis, those with a significant difference in blood pressure between the upper and lower limbs, preterm neonates, patients with acquired heart disease, and syndromic or critically ill patients were excluded from this study. Results: Of the patients in the database, 245 met the inclusion criteria. The median age was 7 months (1 day to 12 years old), and the median weight was 7.85 kg (1.9–54 kg). A normal Abstract for SHA22 195