Clinical impact of pulmonary arterial hypertension on SARS-CoV-2 outcomes: U.S. pre-vaccination analysis.

Our retrospective study aimed to determine how pulmonary arterial hypertension (PAH) influences the clinical outcomes of COVID-19 admissions by using data from the 2020 nationwide inpatient sample (NIS). Among the 1,018,915 adults who were hospitalized with COVID-19 in 2020, 155 also had a PAH diagnosis. After adjusting for all baseline demographics and co-morbidities through multivariate analysis, we found that in patients admitted with a principal diagnosis of COVID-19, PAH was not associated with an increased risk of mortality compared to those without PAH. (adjusted OR 0.58 [95% CI 0.2-1.6] p=0.3). In addition, patients with both COVID-19 and PAH showed no statistically significant difference in the odds of requiring mechanical ventilation (adjusted OR 1.1 [95% CI 0.5-2.6] p=0.9), vasopressor needs (adjusted OR 0.4 [95% CI 0.1-3.5] p=0.4), acute kidney injury necessitating renal replacement therapy(adjusted OR 0.7 [95% CI 0.3-1.7] p=0.5), mean length of stay (LOS) (11.1 vs. 7.5 days), adjusted difference 3.1 [95% CI -3.8- 10.1] p=0.37) or mean total hospitalization charges ($195,815 vs $79,082, adjusted difference 107,146 [95% CI -93,939 - 308,232] p=0.29). Further studies are needed to investigate this subpopulation during the post-vaccination era to observe the effects of outcomes in these patients.

Multicenter Experience for Early and Mid-Term Outcome of MyVal Transcatheter Pulmonary Valve Implantation.

Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has recently been reported to be implanted in pulmonary position. Myval transcatheter valve were implanted in patients with stenosed dysfunctional conduits, severe regurgitation from transannular patch or dysfunctional surgical pulmonary valves (Bioprosthesis). Procedural details and post-TPVI follow-up were analysed. Myval TPVI was used in Fifty three patients with median age of 15 years (IQR 12-19.5 years). Almost sixty percent of the patients were male, with a median weight of 50 kg (31-63 kg). Prestenting was used in more than 80 percent of patients (n = 45 patients), while 6 patients had a prior surgical valve implantation. After Myval TPVI implantation, the peak instantaneous gradient across the RVOT decreased from a median of 23.5 mmHg (IQR 10-53 mmHg) pre-procedure to 10 mmHg (IQR 5-16 mmHg) post-procedure. The median fluoroscopy time for the procedure was 35 min (IQR 23.5-44 min). The large sizes-mainly the 29-mm and 32 mm Myval (Navigator, Meril Life Sciences Pvt Ltd, India), were the most used size in 40% (n = 22) of the cases each. The median contrast volume used during the procedure was 247 mL (IQR 120-300 mL). Patients were followed for a median period of 360 days (IQR 164-525 days). At the last clinic follow-up, there was no tricuspid valve regurgitation. Moderate neo-pulmonary valve regurgitation was reported in three cases. Early experience of TPVI with MyVal is encouraging with procedural success in all patients and acceptable mid-term outcomes.

Clinical Outcomes of Decompensated Heart Failure With Reduced Ejection Fraction Admissions With or Without Atrial Fibrillation and Atrial Flutter.

The aim of our retrospective study is to determine the influence of co-morbid atrial fibrillation or flutter (AF) on decompensated congestive heart failure (CHF) admissions using data from the 2020 nationwide inpatient sample. We identified 76,835 adults admitted nonelectively with decompensated CHF. After multivariate adjustment, we found decompensated heart failure with reduced ejection fraction (HFrEF) admissions with AF had 37% higher odds of in-hospital mortality, (OR 1.38 [95% CI 1.1-1.72] P < 0.01), 33% higher odds for mechanical ventilation (MV) (OR 1.33 [95% CI 1.14-1.55] P < 0.01), 39% higher odds of early MV (OR 1.39 [95% CI 1.16-1.66] P < 0.01), 54% higher odds of cardiogenic shock (OR 1.54 [95% CI 1.29-1.84] P < 0.01), 61% increased odds of mechanical circulatory support (MCS) requirement (OR 1.61 [95% CI 1.12-2.31] P < 0.02), significantly higher odds of acute renal failure (AKI) necessitating dialysis (OR 2.20 [95% CI 1.39-2.48] P < 0.01), 1-day increase in mean length of stay (LOS) (6.7 vs 5.7 days, adjusted difference: 0.99, P < 0.01), $13,281 increase in total hospitalization charges ($84,316 vs $74,279, adjusted difference: $13,281, P < 0.05) compared to the non-AF cohort. Moreover, we found decompensated heart failure with preserved ejection fraction (HFpEF) admissions with AF had a 23% increased odds of MV (OR 1.23 [95% CI 1.01-1.50] P < 0.01), 24% higher odds of early MV (OR 1.24 [95% CI 1.00-1.53] P < 0.01), 0.36 days increase in mean LOS (5.5 vs 5.2 days, adjusted difference: 0.36, P = < 0.01), but no significant difference in in-hospital mortality (OR 1.23 [95% CI 0.86-1.75] P = 0.25), cardiogenic shock (OR 1.75 [95% CI 0.96-3.19] P < 0.07), dialysis-dependent AKI (OR 0.46 [95% CI 0.18-1.17] P < 0.10), or mean total hospitalization charges ($52,086 vs $47,990, adjusted difference: $5584, P = 0.06) compared to the non-AF cohort.

Clinical Outcomes of Heart Transplant Recipients Admitted with COVID-19 Infection in 2020: A Nationwide Analysis.

The COVID-19 pandemic, caused by infection of the SARS-CoV-2 virus, has impacted morbidity and mortality through widespread cytokine release and aberrant immunity; the mainstay of management has been immunosuppression. The aim of our retrospective study is to determine the effects of solid-organ transplantation (SOT) on COVID-19 admissions using data from the 2020 nationwide inpatient sample (NIS). After multivariate adjustment, we found COVID-19 admission with SOT had no difference in mortality (11.5% vs 11.1%, adjusted OR: 0.99 [95% CI 0.84-1.19, P = 0.99], no difference in need for vasopressor use (2.6% vs 1.8%, adjusted OR: 1.02 [95% CI 0.73-1.44, P = 0.88]), lower odds of requiring mechanical ventilation (MV) (13.7% vs 14.8%, adjusted OR: 0.83 [95% CI 0.71-0.97, P = 0.02]), lower odds of MV within 24 hours of admission (adjusted OR: 0.60 [95% CI 0.47-0.78, P < 0.01]), increased odds of mechanical circulatory support needs (adjusted OR 3.7 [95% CI 1.2-11.7, P = 0.025]), increased odds of acute renal failure requiring renal replacement therapy (adjusted OR 1.66 [95% CI 1.29-2.15, P < 0.01]), decreased mean length of stay (7.45 days vs 7.48 days, adjusted difference: 0.8 days less, P <0.01), and no difference in mean total hospitalization charges ($91,316 vs $79,100, adjusted difference: -$2,667, P = 0.57) compared to COVID-19 admissions without SOT.

Irritable Bowel Syndrome Symptoms in Nonalcoholic Fatty Liver Disease Patients Are an Indicator of Depression and Anxiety.

GOALS: Determine factors associated with Irritable bowel syndrome (IBS) in nonalcoholic fatty liver disease (NAFLD) patients. BACKGROUND: 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. STUDY: 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. RESULTS: 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%; P <0.001). CONCLUSION: 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.

Short- and medium-term outcomes for patent ductus arteriosus stenting in neonates ≤2.5 kg with duct-dependent pulmonary circulation.

BACKGROUND: Morbidity with surgical systemic-to-pulmonary artery shunting (SPS) in infants ≤2.5 kg has remained high. Patent ductus arteriosus (PDA) stenting may be a valid alternative. The objective of this study is to evaluate outcomes following PDA stenting in patients ≤2.5 kg from four large tertiary centers. METHODS: Retrospective review of all neonates ≤2.5 kg with duct-dependent pulmonary circulation who underwent PDA stenting. Procedural details, pulmonary arterial growth, reinterventions, surgery type, and outcomes were assessed. RESULTS: PDA stents were implanted in 37 of 38 patients attempted (18 female) at a median procedural weight of 2.2 kg (interquartile range [IQR], 2-2.4 kg). Seven patients (18%) had a genetic abnormality and 16 (42%) had associated comorbidities. The median intensive care unit stay was 4 days (IQR, 2-6.75 days), and the median hospital stay was 20 days (IQR, 16-57.25). One patient required a rescue shunt procedure, with three others requiring early SPS (<30 days postprocedure). Twenty patients (54%) required reintervention with either balloon angioplasty, restenting, or both. At 6-month follow-up, right pulmonary artery growth (median z-score -1.16 to 0.01, p = 0.05) was greater than the left pulmonary artery (median z-score -0.9 to -0.64, p = 0.35). Serious adverse effects (SAEs) were seen in 18% (N = 7) of our cohort. One patient developed an SAE during planned reintervention There were no intraprocedural deaths, with one early procedure-related mortality, and three interstage mortalities not directly related to PDA stenting. CONCLUSIONS: PDA stenting in infants ≤2.5 kg is feasible and effective, promoting pulmonary artery growth. Reintervention rates are relatively high, though many are planned to allow for optimal growth before a definitive operation.

Outcome of ventricular septal defect closure with the Nit-Occlud® Le VSD-Coil: single centre experience.

The objective of this study was to review the outcome of patients who underwent transcatheter closure of ventricular septal defect (VSD) using Nit-Occlud® Leˆ VSD-Coil.A retrospective study was carried out at King Abdulaziz Cardiac Center on all paediatric patients who underwent interventional VSD closure, during the period from 2011 and 2016. Data were collected, including demographic information, cardiac diagnosis, pre-operative diagnosis, intra-operative findings and postoperative events. Out of 46 patients, who were evaluated for VSD device closure, 17 of them were excluded; 24 patients out of 29 had successful closure of VSD using Nit-Occlud Leˆ VSD-Coil (success rate of 83%), and five cases were referred to surgery. The age group was from 2 years up to 18 years. Almost 90% of VSD was the perimembranous (PM) type (26 patients) and 3 patients (10%) with muscular VSD. Ten of the 26 PM VSDs had TV accessory tissue partially covering the VSD defects. The hemodynamic assessment showed pulmonary blood flow to systemic blood flow ratio (Qp:Qs) > 1.5 in almost all the patients who underwent defect closure. Immediate closure of the defect was achieved in 11/24 patients (45%). In five additional patients, there was a tiny residual and eight with a small residual. With 6 months of follow-up, only six patients remained with tiny residual VSD defects.The Nit-Occlud® Leˆ VSD-Coil device has minimal effects on the aortic and tricuspid valves, and there was no permanent atrioventricular block, especially in patients with PM VSDs. Patients with a residual shunt should be followed, especially for haemolysis.

Late outcomes of surgical reconstruction of peripheral pulmonary arteries.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Pulmonary Vascular Resistance Measurement Remains Keystone in Congenital Heart Disease Management.

Pulmonary vascular resistance (PVR) plays a major role in congenital heart management and critical decision. The impact of pulmonary vascular disease in the early and late morbidity and mortality after cardiac surgery and interventional catheterization in congenital heart defect (CHD) highlights the importance of critical evaluation for PVR. Currently, PVR is evaluated with invasive cardiac catheterization for hemodynamic data collection, processing, and analysis. Despite the limitation of hemodynamic evaluation in the setting of CHD, accurate data analysis, and interpretation have significant impact on clinical outcome and procedure success. This article reviews the basic calculation of PVR in the setting of congenital heart disease with diagrammatic illustration for easy understanding of the hemodynamic.

Pulmonary arteriovenous malformations in children after the Kawashima procedure: Risk factors and midterm outcome.

BACKGROUND: Pulmonary arteriovenous malformations (PAVMs) are the major cause of progressive cyanosis in patients palliated with bidirectional cavopulmonary connection (BCPC). The aim of our study is to analyze the occurrence of PAVMs in patients after Kawashima procedure, to study the effect of total cavopulmonary connection (TCPC) on PAVMs, to evaluate the effect of axillary arteriovenous fistula (AAVF) creation on PAVMs, and to study the risk factors for PAVMs. METHODS: In this retrospective cohort study, all patients with left isomerism and azygous continuation of an interrupted inferior vena cava who underwent Kawashima procedure from July 2001 to December 2017 were included. RESULTS: Twenty.six patients after Kawashima procedure were included in our study. PAVMs were diagnosed in 12 patients (46%). Five of these 12 patients underwent TCPC with complete resolution of hypoxemia. Three patients underwent AAVF creation, 2 had complete resolution, while 1 had partial resolution of hypoxemia. Fourteen patients (54%) did not develop PAVMs. Nakata index below 267 mm2/m2and McGoon ratio below 1.9 predicted the development of PAVMs with high sensitivity and specificity. CONCLUSIONS: PAVMs represent a serious complication in patients who undergo Kawashima procedure. Small size of pulmonary arteries is an important risk factor for the development of PAVMs. Resolution of hypoxemia after TCPC completion supports the hepatic factor hypothesis. Early TCPC completion in these patients may help to avoid the development of PAVMs by restoring the hepatic factor. Resolution of hypoxemia after AAVF creation may support the lack of pulsatile flow hypothesis.

Stent or Shunt, What Could be Better for Children with Duct Dependent Pulmonary Circulation?

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.

Pediatric Cardiologist's View on Centralization of Pediatric Cardiology Services: A Questionnaire Survey.

The population of the Kingdom of Saudi Arabia (KSA) exceeding 35 million people and in the presence of a non-structured increase in the number of pediatric cardiac centers, we expect to face some concerns like dilution of the service where the volume will be less than the acceptable standards, the increase in mortality and morbidity, and failure to obtain personalized medicine at a reasonable cost. Therefore, we built up this survey questionnaire about those concerns and collected the opinion of expert medical staff in Saudi Arabia who are working in the field of pediatric cardiology. Seventy percent of the responders vs. 25% recommend the centralization of the PCS as the solution for the above concerns, and 94% recommend sticking to the globally accepted criteria when issuing the license of the centers providing PCS including the volume of patients in each center, and minimum multidisciplinary facilities in terms of resources, services, and personnel.

Percutaneous Left Axillary Artery Placement of Intra-Aortic Balloon Pump in Advanced Heart Failure Patients.

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.

Percutaneous balloon dilatation for congenital aortic stenosis during infancy: A 15-year single-center experience.

BACKGROUND: Congenital aortic stenosis (AS) is a rare disease. Treatment options for newborns are challenging. Newborns may have higher reintervention rate and mortality. OBJECTIVES: The study aimed to identify the factors predictive of reintervention following balloon aortic valvuloplasty (BAV) for AS during infancy. METHODS: Retrospectively, between 2001 and 2016, echocardiography (echo) and cardiac catheterization data for infants with AS were analyzed, including follow-ups and reinterventions. Percentage reduction was defined as the ratio between the drop of aortic valve (AV) peak gradient and the baseline peak gradient. RESULTS: Sixty infants were included and 48 were followed up. Sixteen (27%) patients were neonates. Peak-to-peak gradient at AV was 64 ± 27 mmHg, which was reduced to 27 ± 13 mmHg. Percentage reduction was 53% ±24%. Forty-nine (82%) patients had adequate results (residual AV gradient <35 mmHg). There was no significant aortic insufficiency (AI) before procedure, while 6 (10%) patients had increased AI immediately after BAV. Of 48 patients, 14 (29%) required an additional BAV. Of 48 patients, 8 (17%) required surgical interventions following BAV. Reintervention was associated more with small left ventricular outflow tract (LVOT), high residual AV, and low percentage reduction. Mortality was 8.3%. CONCLUSIONS: BAV in infancy has a reasonable success rate (82%) with high rate of reintervention. Patent ductus arteriosus-dependent neonates carried the highest risk of mortality. Small LVOT, high AV residual gradient, and low percentage reduction resulted in more reinterventions.

Pulmonary arteriovenous malformation in a neonate: a condition commonly misdiagnosed.

Pulmonary arteriovenous malformations (PAVMs) are congenital defects in the 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, leftover 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 PAVM in a neonate, which was done on two stages with a 6 months interval, with no complications. Over 2-year follow-up, the patient remained well with no symptoms.

Pulmonary hypertension and vasculopathy in incontinentia pigmenti: a case report.

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 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.

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.