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Background Systemic to pulmonary shunt (Shunt) is offered for children with duct dependent pulmonary circulation to augment pulmonary flow. Recently patent ductus arteriosus (PDA) stent (Stent) is widely used as an alternative method. We aimed to compare post intervention outcomes in children underwent either procedure. Methods Infants under 3 months who had an initial palliation by Shunt or Stent were retrospectively reviewed between 2008 and 2016, then followed till the second intervention or 1 year whichever earlier. Results 187 patients (110 Shunt and 77 Stent) were included. Initial weight and pulmonary artery (PA) branches size were similar between the groups. Shunt patients had more shock preoperatively and required more emergency intervention. Stent group showed less ICU stay 4 (1–8) vs 13 (7–23) days, p < 0.0001 and less positive pressure ventilation days 1 (0–2) vs 5.5 (3–11), p < 0.0001. However, Stent group had more symptomatic arterial and deep venous thromboses. In Stent patients the branch PAs growth was better and more homogeneous. At follow-up, no difference between groups regarding cumulative readmission days to hospital, hemoglobin levels and the weight percentile for age. Mortality was not different with a tendency to be higher in the Shunt group (13%) compared to the Stent group (5%), p 0.1. Conclusions The implantation of PDA stent in patients with duct dependent pulmonary circulation results in a smoother ICU course and a shorter hospital stay, with higher risk of vascular injury. Shunt and Stent procedures have a good outcome for PA growth, somatic growth and survival.

Determine factors associated with Irritable bowel syndrome (IBS) in nonalcoholic fatty liver disease (NAFLD) patients.IBS affects 10% to 15% of the adult population worldwide and is linked to anxiety and depression. The impact of IBS-type symptoms in NAFLD patients is not well described.A cross-sectional study of patients in the hepatology clinic at Houston Methodist Hospital was performed based on a respondent postal survey. IBS was defined by the Rome IV questionnaire, anxiety and depression were assessed with the Hospital Anxiety Depression scale. Patients with inflammatory bowel disease, colorectal carcinoma, or small bowel tumors were excluded. Patients were divided based on Rome IV diagnostic criteria for IBS and Hospital Anxiety Depression scale.130 patients were included in the analysis, 38 satisfied Rome IV criteria for IBS (IBS group) versus 92 who did not (non-IBS group). Depression was more prevalent in the IBS group (18.4% vs 5.4%, P=0.01). Anxiety was also greater in the IBS group (31.6% vs 9.8%, P=0.002). Female sex, depression, and body mass index (BMI)30 were independent predictors of IBS in NAFLD in 4 multiple logistic regression models. In newly diagnosed IBS patients, gamma-glutamyl transferase levels were lower (67.5 vs 28, P=0.04). Current abdominal pain was higher than 100% versus 81.3% (P=0.045), as was pain associated with the change in stool frequency (96.3% vs 50%; P0.001).Our study highlights the increased rate of IBS symptoms, depression, and anxiety in patients with NAFLD. Clinicians should be alert when IBS symptoms are reported by a NAFLD patient and be aware of the impact of these comorbidities on quality of life and response to therapy.

Objectives This study presents the largest clinical experience of percutaneously placed axillary intra-aortic balloon pump (IABP) in patients with advanced heart failure. Background Transfemoral placement of IABP limits mobility and recuperation in patients who need prolonged support. We had previously reported a novel percutaneous method of IABP placement in the axillary artery and now present our expanded experience with this technique. Methods We performed a retrospective chart review of patients with advanced heart failure with percutaneous axillary IABP placement from November 2007 to June 2018 at Houston Methodist Hospital. We defined successful cardiac replacement therapy as heart transplant or left ventricular assist device implantation. We compared patients who had successful cardiac replacement with those who died and those who needed unplanned escalation of mechanical circulatory support. Results Of the 195 patients identified, 133 (68%) underwent successful cardiac replacement (120 transplants and 13 left ventricular assist device) as planned. End-organ function improved on IABP support in patients bridged to next therapy. There were 16 patients that died while on IABP support and 18 needed escalation of support. Higher right atrial/wedge ratio, higher right atrial pressure, smaller left ventricular end diastolic dimension, and ischemic cardiomyopathy were associated with death on the IABP in multivariate analysis. Post-transplant and post left ventricular assist device survival for those bridged successfully was 87% and 62%, respectively. Although bedside repositioning was frequent, 37% needed replacement for malfunction. Vascular complications occurred in a minority. Conclusions Percutaneous axillary approach for IABP placement is a feasible strategy for prolonged mechanical circulatory support in patients with advanced heart failure.

Cardiac tamponade as a result of pericardial effusion (PE) is a serious uncommon condition in the neonatal period. PE in such cases could be associated with hydrops fetalis, neonatal sepsis, metabolic diseases, or as a complication of percutaneous indwelling central catheter. 1 We are reporting a preterm baby, with low birth weight who developed large PE as a complication of total parenteral nutrition via a peripherally inserted central catheter, managed successfully with pericardiocentesis.

The ideal management of peripheral pulmonary artery stenosis is still controversial. We adopted a primary surgical approach to this complex lesion with excellent early outcomes. In this study, we analyzed our late outcomes.We performed a retrospective review of 91 patients with biventricular anatomy who underwent peripheral pulmonary artery reconstruction from March 2008 to July 2020. Our surgical approach included either a single-stage complete repair through median sternotomy or a 2-stage repair through sternotomy/left thoracotomy, depending on the degree of distal involvement of the left pulmonary artery branches.Median age was 26 months. Syndromic etiology was established in 54 patients (59.3%) versus nonsyndromic etiology in 37 patients (40.7%). Single-stage repair was achieved in 68 patients (74.7%). There were 2 (2.2%) in-hospital mortalities. The mean right ventricular to aortic systolic pressure ratio decreased from 1.07 ± 0.20 preoperatively to 0.32 ± 0.07 immediately postoperatively (P .001), representing a 70.1% reduction. At 1-year postoperative catheterization, the mean right ventricular to aortic systolic pressure ratio was 0.28 ± 0.05 (P .001 compared with immediately postoperative value). With a median follow-up of 68 months (IQR, 39-117.5 months), there was no late mortality after discharge. All patients were active and asymptomatic on the most recent follow-up. There were no early or late reinterventions on pulmonary arteries.Late outcomes of surgical reconstruction of peripheral pulmonary arteries are excellent and durable in various pathologies (syndromic and nonsyndromic) with a significant reduction in right ventricular to aortic systolic pressure ratio, low mortality, and no reintervention.

Incontinentia pigmenti (IP; Bloch–Sulzberger syndrome) is a rare, genetic syndrome inherited as an X-linked dominant trait. It primarily affects female infants and is lethal in the majority of males during fetal life. The clinical findings include skin lesions, developmental defects, and defects of the eyes, teeth, skeletal system, and central nervous system. Cardiovascular complications of this disease in general, and pulmonary hypertension in particular, are extremely rare. This report describes the case of a 3-year-old girl with IP complicated by pulmonary arterial hypertension. Extensive cardiology workup done to the patient indicates underlying vasculopathy. This report sheds light on the relationship between IP and pulmonary hypertension, reviews the previously reported cases, and compares them with the reported case.

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.

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.

Pulmonary arteriovenous malformations (AVM) are congenital defects in a form of an anomalous bridging between a pulmonary arterial and venous system that sidesteps the normal pulmonary capillary. This anomaly is usually associated with hereditary hemorrhagic telangiectasia (HHT), left over small group are sporadic cases, but may occur as an isolated anomaly or as multiple lesions. Rarely, such abnormalities can be acquired. Few cases are diagnosed in the neonatal period, as it will pass silent without symptoms. Clinical manifestations occur soon after birth as cyanosis, tachypnea, and an abnormal sound as bruit over the AVM; and the most important point is the clinical suspicion of diagnosis in absence of pulmonary parenchymal and cardiac disease that can explain the persistence of cyanosis. We report a successful Amplatzer vascular plug occlusion of isolated multiple left side pulmonary AVM in a neonate, which was done on two stages with a 6 months interval, with no complications. Over two-year follow up the patient remained well with no symptoms.Pulmonary arteriovenous malformations (PAVM) are congenital defects in a form of an anomalous bridging between a pulmonary arterial and venous system that sidesteps the normal pulmonary capillary, this anomaly usually associated with hereditary hemorrhagic telangiectasia (HHT), left over small group are sporadic cases, but may occur as an isolated anomaly or as multiple lesions. Rarely, such abnormalities can be acquired. Few cases are diagnosed in neonatal period as it will pass silent without symptoms. Clinical manifestations soon after birth in a form of cyanosis, tachypnea, and an abnormal sound as bruit over the AVM, and the most important point is the clinical suspicion of diagnosis in absence of pulmonary parenchymal and cardiac disease that can explain the persistence of cyanosis. We report a successful Amplatzer vascular plug occlusion of isolated multiple left side PAVM in a neonate, which was done on two stages with 6 months interval, with no complication and during two years follow up, patient is doing fine with no symptoms.

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.

Aim: Infantile scimitar syndrome is a rare condition, with most of the literature reports being limited to case reports and a few case series. The aim of this study was to review patients with infantile scimitar syndrome who presented to our hospital from July 2000 to January 2011. Materials and Methods: In this retrospective study, we evaluated the medical records of patients aged 0-14 years who were symptomatic before the age of 1 year and were subsequently diagnosed with the syndrome. A total of 16 patients with the infantile form of scimitar syndrome were identified from the database. Results: The median age at presentation was 14 days, with a median age at diagnosis of 55 days. Fifty-six percent of the patients were females. Tachypnea was the major presenting symptom and 13 out of 16 patients had pulmonary hypertension. Of the 13 patients with pulmonary hypertension, 7 had systemic collaterals, which were treated by coil occlusion together with medications, and 3 had corrective surgery. The mortality rate was 3/16 (18.8%) over the 10.5 years study period. Conclusions: Infantile scimitar syndrome is a rare congenital anomaly that needs a high degree of suspicion for early referral and management. The association of the syndrome with pulmonary hypertension leads to recurrent and prolonged hospitalization.

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.

Introduction: The aim of this study was to identify factors predictive of reintervention following balloon aortic valvuloplasty (BAV) for congenital aortic stenosis (AS) during infancy. Methodology: Retrospectively, patients with congenital AS who required BAV in the first year of life between 2001 and 2016 were included. Patients who required direct surgical intervention were excluded. Echocardiography (echo) and cardiac catheterization (cath) data were analyzed. Response ratio (RR) was defined as the ratio between the drop in aortic valve (AV) peak gradient to the baseline peak gradient. Follow-ups and the need for reintervention were documented. Results: A total of 60 infants were included. Sixteen patients (27%) were newborns. The average peak-to-peak gradient from the left ventricle to the ascending aorta was 64 ± 27 mmHg, which was reduced to an average of 27 ± 13 mmHg. Average response ratio was 53 ± 24%. None of the patients had a significant aortic insufficiency (AI) before procedure, while 6 (10%) had significant AI immediately after BAV. Fourteen patients (23%) required an additional BAV. They had low cath-RR of 39 ± 27 and echo-RR of 15 ± 33 versus those who had BAV once with ratios of 60 ± 17 (cath; p = 0.002) and 36 ± 28 (echo; p = 0.025) during the initial intervention. Eight patients (13.3%) required surgical interventions following BAV. They had small AV to pulmonary valve (PV) ratios (p = 0.029) and higher residual AV gradients (p

Introduction We had previously published a percutaneous technique of axillary intra-aortic balloon pump (IABP) placement in patients being bridged to transplant for facilitation of ambulation and physical recovery. However, this enhanced mobility of the patient may predispose to a higher risk of IABP malposition with unique complications compared to the non-ambulatory state of femoral IABP therapy. We present our experience to illustrate this. Methods We conducted a retrospective chart review of patients that received percutaneous axillary IABP at our hospital from October 2015 to February 2018. Results A total of 75 patients had percutaneous axillary IABP insertion during the study period. Of these, 2 had gross IABP malposition into the arch of aorta and 5 patients had superior mesenteric artery (SMA) occlusion by IABP. Table 1 describes the specifics of these patients. Conclusion There has been a wealth of experience with femoral IABP use wherein the malpositions described above have not been reported. As utilization of IABP in the axillary position to promote ambulation is increasing, it is imperative to understand and expect such unique complications for prompt diagnosis and management.

Ross procedure is a surgical option for congenital aortic valve stenosis in young patients. Overtime, patients may develop RV-PA conduit degeneration with pulmonary regurgitation (PR) needing re-intervention. Re-intervention with transcatheter pulmonary valve (TPV) is a feasible option. 38M with

Ross procedure is used in young adults with congenital aortic stenosis. Over time, patients may develop autograft insufficiency and RV-PA homograft dysfunction. 42F with congenital aortic stenosis s/p aortic valvotomy followed by percutaneous valvuloplasty and Ross procedure presented with

Adults with repaired TOF may face sequelae like residual RVOT stenosis and pulmonary regurgitation (PR) that warrant re-intervention. Optimal timing of re-intervention is uncertain. 21F with Trisomy 21, complete AV canal defect and TOF s/p complete repair presented with worsening dyspnea on

The pediatric cardiology division at the King Abdulaziz Cardiac center is a unit that provides a tertiary level of care for children with complex congenital and acquired heart diseases from all around Saudi Arabia. Further, we are also involved in training and research programs, both at national and international levels. This paper describes the organization and metrics of our section as well as our teaching and research activities. Besides our Pediatric Cardiology Fellowship Program, the following additional topics are outlined in detail: outpatient services, echocardiography, advanced cardiac imaging, cardiac catheterization, pediatric electrophysiology, pulmonary hypertension program, data collection, and academic activities.

Introduction Several studies have shown lack of benefit of intra-aortic balloon pump (IABP) in the setting of ST-elevation myocardial infarction and cardiogenic shock. However, IABP continues to be used as mechanical circulatory support for bridge to cardiac transplant in patients with acute-on-chronic heart failure. We conducted this study to analyze characteristics that predict successful bridge to transplant with IABP. Methods We did a single-center retrospective analysis of all patients that received percutaneously inserted axillary IABP as BTT from November 2007 to February 2018. Baseline hemodynamic parameters were assessed by right heart catheterization reports and echocardiograms prior to the implantation of IABP. We calculated left ventricular cardiac power output (W) as mean arterial pressure x cardiac output/451. Death on IABP support was defined as any death occurring while on the waitlist for cardiac transplant and on IABP support. We compared the survivors versus non-survivors using Chi-square test for categorical variables and Student's T-test for continuous variables. We did logistic regression analysis on those variables with statistically significant difference (p-value less than 0.05), followed by multivariable analysis. Results Out of 156 patients that had axillary IABP as BTT- 6 patients (3.8%) died while on IABP support whereas 136 (86.5%) patients were successfully bridged to transplant. The remaining 15 patients required escalation of therapy in the form of Impella (5 patients), left ventricular assist device (7 patients) and total artificial heart (3 patients). Patients that died on IABP support had higher right atrial pressure (RAP) and lower left ventricular end diastolic internal diameter (LViDd) compared to patients that survived. The results are shown in Table 1 and Table 2 . Conclusion Our study shows that patients that survived on axillary IABP support had significantly different RAP and LViDd compared to the patients that died. Interestingly, we also found that cardiac power was not predictive of clinical success in these patients.

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.

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.

The association between congenital major urinary tract anomalies (CMUTA) and spontaneous pneumothorax in term newborn infants (SPTNI) is controversial. We conducted a case-control study to test the hypothesis that SPTNI is associated with CMUTA. We compared 80 term infants with spontaneous pneumothorax to 80 healthy control infants. We recorded risk factors, clinical course, therapy, and outcome. Only 1 infant of 60 infants (1.7%) had CMUTA with SPTNI, as revealed by renal ultrasound studies. This is comparable to the 1.4% rate reported for CMUTA in healthy newborn infants by Steinhart et al. ([1988] Pediatrics 82:609-614). SPTNI were significantly more likely in males with higher birth weights and with vacuum delivery. Sixty-seven (84%) infants with SPTNI had follow-up for a mean and median of 46.4 and 39 months, respectively (range, 1-126 months), without manifesting any renal or pulmonary complications.

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.

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.

Objectives: Describe the short and midterm outcome and to determine the predictors of reintervention in neonates with critical pulmonary stenosis (PS) or pulmonary atresia with intact ventricular septum (PA/IVS). Background: The transcatheter intervention for critical PS and PA/IVS resulted in improvement in the patient's survival and the quality of life. The procedure is not free of complications and there is still a significant rate of reintervention. Method: All neonates with critical PS or PA/IVS who underwent interventional cardiac catheterization between November 2004 and January 2009 were reviewed retrospectively. We performed a comparison between those who required reintervention and those who did not, to identify the predictors of reintervention. Results: Forty-three neonates were included, 23 (53.5%) had critical PS and 20 (46.5%) had PA/IVS. Twenty-six patients (60%) were males, the mean age was 11 ± 8 days, and the mean weight was 3.2 ± 0.6 kg. Two patients died (4.6%). The mean follow-up period was 19 ± 13 months for 42 patients. Fifteen patients (36%) required reintervention, 11 of them (73%) had PA/IVS, and 4 (27%) had critical PS. Reintervention was more in patients with PA/IVS than those with critical PS (P = 0.003). Other predictors for reintervention included hospital stay ≥ 7.5 days (P = 0.001) and tricuspid valve regurgitation peak gradient in day one post first intervention (TR1) ≥ 43 mm Hg (P = 0.03). Conclusion: Interventional cardiac catheterization shows favorable outcome for patients with critical PS and PA/IVS. Predictors for reintervention included the diagnosis of PA/IVS, hospital stay ≥7.5 days after first intervention and TR1 gradient ≥ 43 mm Hg. © 2011 Wiley Periodicals, Inc.

Arterial Tortuosity Syndrome (ATS) is an autosomal recessive connective tissue disease that can present with complex pulmonary arterial stenosis, causing right ventricular (RV) hypertension and dysfunction. In this study, we review our experience and early outcomes with single stage surgical repair.From 2008 to 2010, 7 patients with ATS and severe bilateral pulmonary artery stenosis underwent surgical repair that involved extensive reconstruction of the pulmonary arterial tree bilaterally, including the central, lobar and segmental branches. The clinical records of these patients were reviewed for preoperative data, repair techniques and early clinical outcomes, including reinterventions and recent echocardiographic studies.All 7 patients had bilateral peripheral and central pulmonary artery stenosis with systemic to supra-systemic RV pressure and severe RV dysfunction. Median age at the time of operation was 72 months (range=7 to 120 months). All patients had low RV pressure (≤40% systemic) after the procedure with significant improvement in RV function to normal before hospital discharge. The mean right ventricular/left ventricular pressure ratio decreased from 1.27±0.19 to 0.31±0.06 postoperatively (p0.001). There was no mortality. All patients continued regular follow-up at our institution for a mean follow-up period of 17.6±9.1 months. All patients were alive and asymptomatic with normal RV function and no evidence of elevated RV pressure on most recent echocardiography.Complete surgical reconstruction of the pulmonary arterial tree in advanced cases of ATS is a successful intervention with excellent early clinical and hemodynamic outcomes.

We report a unique case of infantile form of Scimitar syndrome with a patent ductus arteriosus and pulmonary hypertension. The PDA was closed percutaneously using the new Amplatzer Ductal Occluder device type II.

The cardiac catheterization laboratory (cathlab) at King Abdulaziz Cardiac Center is a tertiary facility with the staff and technology to support advanced coronary, valvular, and structural heart disease therapies. The latest interventional devices and supporting diagnostic tools are in routine use. The facility has three biplane laboratories, which serve more than 2000 patients each year. Interventions are well supported by high-quality biplane flat panel imaging, intravascular ultrasound, fractional flow reserve, …

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.

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.

Introduction Over the last decade, stent implantation has become a widely accepted, effective therapy for the treatment of pulmonary arterial (PA) stenoses. Stent implantation has proved to be a safe procedure with minimal complications. Objectives To review our experience with branch PA stenting. We reviewed also catheter based interventions for patients with right aortic arch and crossed pulmonary arteries associated with branch PA stenosis. Method Retrospective data analysis of all patients who had branch PA stenting at KACC, NGHA, Riyadh KSA, during the period from January 2006 till October 2009. Patients with pulmonary atresia VSD and MAPCAS were excluded. Results Twenty-one patients were identified to have PA stenting during the study period. 14 were females (66.7%). Six patients (29%) were syndromic (Alagile syndrome, DiGeorge syndrome, Down syndrome, Goldenhar Syndrome, and Noonan Syndrome). Five patients (24%) had crossed Pas. Seven patients (33%) had PA, VSD, PDA, Two had TOF, one had AVSD with TOF, one DORV, one TGA, one truncus arteriosus with IAA type B, and one had Bicuspid AV with aortic stenosis as a primary diagnosis. All of them had branch PA stenting as a secondary procedure (post surgical intervention). Six patients (29%) had PA stenting as a primary intervention with no prior surgical intervention. Six patients (29%) had Bilateral branch PAs stenting, 11 (52%) had LPA stenting and 4 (19%) had RPA stenting. The median age at branch PA stenting was 35 months (range 2 months to 45 years). The median weight was 10.5 kg (2.8–100 kg). The median F/U period was 32 months (40 days to 61 months). Ten patients (48%) required stent dilatation after a median period of 28 months (12–38 months). One patient had failed LPA stenting (stent embolized and parked into RPA) who later underwent another intervention with successful LPA stenting. Conclusion Stent implantation has proved to be a safe procedure with minimal complications. Even in those with crossed Pas. Restenoses is long term concerns which are amenable to redilatation with excellent results.

two stage procedures to correct Fontan Pathology. Left Glenn shunt as first stage then fenestrated Fontan with common atrioventricular valve repair as a second stage. The patient had uneventual post operative recovery and discharge home. However, she was re-admitted few days after discharge with high fever and manifestation of respiratory chest infection. On investigation she was proven to be H1N1positive. The patient respiratory condition deteriorated dramatically within 48 h. She developed acute respiratory failure which required mechanical respiratory support. She also developed a sever heart failure which required significant chemical ion tropic support. Despite all the above measures, the patient condition continue to deteriorate and the Decision was taken to insert the extracorporeal membrane oxygenation (ECMO), as a bridge for respiratory failure recovery. The ECMO cannulation were Veno-Venous. femoral vein as input flow and internal jugular as output flow connected to pediatric oxygenator D905 with 3/8–3/8 circuit We started with one liter flow per minute, 100% FiO2, 5 L gas flow to maintain her core temperature on 36 C. she got hemofiltration as additional device attached to the circuit to help taking out the excessive fluid. As she was on ECMO for long time her creatinine and urea and lactic acid started to be elevated, so we applied dialysis system beside the regular hemofiltration. She was on heparin infusion to maintain the ACT (180–200 s) therefore, we faced bleeding problem from the cannula sides and even from the mouth. Results: The patients were assessed daily for the respiratory and haemodynamic recovery. Chest X-ray were performed daily to check for any parenchymal infiltarate recovery and the patient became H1N1 negative after 10 days. We were able to start weaning from the respiratory support after 23 days and to be weaned from the mechanical ventilation after one month. Patient however, required aggressive dietary and physiotherapy to help her regain her strength. Conclusion: Diligence, persistence and luck were the essence of this case. Early diagnosis and respiratory support may have helped in saving this child. Tracks: Cardiac Perfusion.