Restriction of congenital portosystemic shunt using the modified microvascular plug.

Congenital portosystemic shunts (CPSS) may produce a variety of severe, clinically detrimental presentations. When indicated, closure is recommended; however, if the intrahepatic portal venous system (IPVS) is underdeveloped complete closure may not be possible and may result in severe acute portal hypertension. Staged restriction of CPSS flow by both surgical and complex transcatheter interventions has been successful in augmenting development of the IPVS such that complete occlusion of the CPSS can be performed. We report use of a modified microvascular plug to restrict CPSS flow with subsequent IPVS development and safe complete occlusion of CPSS.

Hot topics in interventional cardiology: Proceedings from the society for cardiovascular angiography and interventions 2020 think tank.

The society for cardiovascular angiography and interventions (SCAI) think tank is a collaborative venture that brings together interventional cardiologists, administrative partners, and select members of the cardiovascular industry community for high-level field-wide discussions. The 2020 think tank was organized into four parallel sessions reflective of the field of interventional cardiology: (a) coronary intervention, (b) endovascular medicine, (c) structural heart disease, and (d) congenital heart disease (CHD). Each session was moderated by a senior content expert and co-moderated by a member of SCAI's emerging leader mentorship program. This document presents the proceedings to the wider cardiovascular community in order to enhance participation in this discussion, create additional dialogue from a broader base, and thereby aid SCAI and the industry community in developing specific action items to move these areas forward.

The influence of Val158Met COMT on physiological stress responsivity.

We tested the extent to which the catechol-O-methyltransferase (COMT) Val158Met polymorphism is associated with stress response and pain in both men and women. The participants were assessed on measures of perceived pain and state/trait anxiety in association with their COMT allele status. We also measured cortisol and salivary alpha-amylase (sAA) levels since previous research suggests an association between the COMT Val159Met polymorphism, cortisol secretion, and sAA activity. We found significant differences between methionine (Met) allele carriers and valine (Val) homozygotes following a stress manipulation in sAA levels. Met allele carriers had a stronger sAA response when compared to Val homozygotes. Furthermore, Val homozygotes showed a positive correlation between their sAA levels and trait anxiety at baseline and 20 min post-stress manipulation but displayed a negative correlation with the change in sAA levels from baseline to 20 min post-stress manipulation. Finally, state/trait anxiety was significantly correlated in Met allele carriers. These findings add support to the COMT warror/worrier model which states under stressful situations, increased dopamine levels in Val (warrior) homozygotes affords an emotional advantage relative to Met (worrier) allele carriers, who show an increased reactivity to aversive stimuli. Summary These findings offer new support for the warrior/worrier model of COMT genotype (rs4680) on human behavior. Twenty minutes following exposure to a cold stress, Val homozygotes (warrior) showed a lower biochemical stress response (salivary alpha-amylase, sAA) relative to methionine (Met) allele carriers (worrier). We further show that the COMT genotype differentially influences state and trait anxiety measures as they relate to stress responses and to each other.

Transcatheter Tricuspid Valve-in-Valve Implantation for the Treatment of Dysfunctional Surgical Bioprosthetic Valves: An International, Multicenter Registry Study.

BACKGROUND: Off-label use of transcatheter aortic and pulmonary valve prostheses for tricuspid valve-in-valve implantation (TVIV) within dysfunctional surgical tricuspid valve (TV) bioprostheses has been described in small reports. METHODS AND RESULTS: An international, multicenter registry was developed to collect data on TVIV cases. Patient-related factors, procedural details and outcomes, and follow-up data were analyzed. Valve-in-ring or heterotopic TV implantation procedures were not included. Data were collected on 156 patients with bioprosthetic TV dysfunction who underwent catheterization with planned TVIV. The median age was 40 years, and 71% of patients were in New York Heart Association class III or IV. Among 152 patients in whom TVIV was attempted with a Melody (n=94) or Sapien (n=58) valve, implantation was successful in 150, with few serious complications. After TVIV, both the TV inflow gradient and tricuspid regurgitation grade improved significantly. During follow-up (median, 13.3 months), 22 patients died, 5 within 30 days; all 22 patients were in New York Heart Association class III or IV, and 9 were hospitalized before TVIV. There were 10 TV reinterventions, and 3 other patients had significant recurrent TV dysfunction. At follow-up, 77% of patients were in New York Heart Association class I or II (P<0.001 versus before TVIV). Outcomes did not differ according to surgical valve size or TVIV valve type. CONCLUSIONS: TVIV with commercially available transcatheter prostheses is technically and clinically successful in patients of various ages across a wide range of valve size. Although preimplantation clinical status was associated with outcome, many patients in New York Heart Association class III or IV at baseline improved. TVIV should be considered a viable option for treatment of failing TV bioprostheses.

Use of 3-D digital subtraction rotational angiography during cardiac catheterization of infants and adults with congenital heart diseases.

OBJECTIVE: To compare image quality, radiation and contrast doses required to obtain 3D-Digital subtraction rotational angiography (3D-DSRA) with 3D-Digital rotational angiography (3D-DRA) in infants (children ≤ 2 years of age) and adults with congenital heart diseases (ACHD). BACKGROUND: 3D-DRA can be performed with radiation doses comparable to bi-plane cine-angiography. However, 3D-DRA in infants requires a large contrast volume. The resolution of 3D-DRA performed in ACHD patients is limited by their soft tissue density. We hypothesized that the use of 3D-DSRA could help alleviate these concerns. METHODS: Radiation (DAP) and contrast doses required to obtain 3D-DSRA was compared with 3D-DRA in 15 age-, size-, and intervention-matched infants and 15 ACHD patients. The diagnostic quality and utility of these two modalities were scored by 4 qualified independent observers. RESULTS: Both in infants and adults, the median contrast volume for 3D-DSRA was lower than 3D-DRA (0.98 vs. 1.81 mL/kg; P < 0.001 and 0.92 vs. 1.4 mL/kg; P < 0.001, respectively) with an increased DAP (median: 188 vs. 128 cGy cm2 ; P = 0.068 and 659 vs. 427 cGy cm2 ; P = 0.045, respectively). The diagnostic quality and utility scores for rotational-angiography, and 3D-reconstruction were superior for 3D-DSRA (score = 94 vs. 80%, P = 0.03 and 90 vs.79%, P = 0.01, respectively) and equivalent for multi-planar-reformation and 3D-roadmapping in ACHD patients compared with 3D-DRA. All scores for both modalities were equivalent for infants. CONCLUSIONS: 3D-DSRA can be acquired using lower contrast volume with a mildly higher radiation dose than 3D-DRA in infants and ACHD patients. The diagnostic quality and utility scores for 3D-DSRA were higher in ACHD patients and equivalent for infants compared with 3D-DRA.

Transcatheter valve implantation for right atrium-to-right ventricle conduit obstruction or regurgitation after modified Björk-fontan procedure.

BACKGROUND: The most common reason for reintervention after a Björk modification of the Fontan procedure, in which the right ventricle (RV) is incorporated into the pulmonary circulation by connecting the right atrial (RA) appendage to the RV directly or with an extra-anatomic graft, is obstruction or regurgitation of the RA-RV connection. Transcatheter implantation of a valved stent is an appealing option for the treatment of RA-RV conduit dysfunction in these patients. In the present study, we assessed early and intermediate results after transcatheter valve implantation within an obstructed or regurgitant RA-RV modified Fontan pathway. METHODS: Through a retrospective multicenter registry, we collected data from 16 patients with a modified Fontan circulation who were treated with percutaneous Melody or Sapien valve implantation for dysfunction of an extra-anatomic RA-RV conduit or valve. RESULTS: All patients had successful and uncomplicated implantation of a Melody (n = 15) or Sapien 3 (n = 1) valve with hemodynamic and, in most cases, clinical improvement. During a median follow-up of 3.3 years, 3 patients died of cardiovascular causes unrelated to the procedure or the valve, and no major valve dysfunction was observed. CONCLUSION: Percutaneous transcatheter valve deployment to treat a dysfunctional RA-RV connection after a Björk modification of the Fontan procedure is a viable alternative to surgery, with low procedural risk, and appears to offer good early and intermediate results.© 2016 Wiley Periodicals, Inc.

Preprocedural Risk Assessment Prior to PPVI with CMR and Cardiac CT.

Percutaneous pulmonary valve intervention (PPVI) is a less invasive and less costly approach to pulmonary valve replacement compared with the surgical alternative. Potential complications of PPVI include coronary compression and pulmonary arterial injury/rupture. The purpose of this study was to characterize the morphological risk factors for PPVI complication with cardiac MRI and cardiac CTA. A retrospective review of 88 PPVI procedures was performed. 44 patients had preprocedural cardiac MRIs or CTAs available for review. Multiple morphological variables on cardiac MRI and CTA were compared with known PPVI outcome and used to investigate associations of variables in determining coronary compression or right ventricular-pulmonary arterial conduit injury. The most significant risk factor for coronary artery compression was the proximity of the coronary arteries to the conduit. In all patients with coronary compression during PPVI, the coronary artery touched the conduit on the preprocedural CTA/MRI, whilst in patients without coronary compression the mean distance between the coronary artery and the conduit was 4.9 mm (range of 0.8-20 mm). Multivariable regression analysis demonstrated that exuberant conduit calcification was the most important variable for determining conduit injury. Position of the coronary artery directly contacting the conduit without any intervening fat may predict coronary artery compression during PPVI. Exuberant conduit calcification increases the risk of PPVI-associated conduit injury. Close attention to these factors is recommended prior to intervention in patients with pulmonary valve dysfunction.

A novel method to prevent recurrent balloon rupture during dilation of heavily calcified conduits in preparation for transcatheter pulmonary valve placement.

INTRODUCTION: A 24-year-old female presented for percutaneous pulmonary valve placement. She was born with tetralogy of Fallot and had initial palliation with a Blalock-Taussig shunt followed by complete repair at age of 4 years including placement of a homograft conduit in the right ventricle to pulmonary artery position. She had developed severe obstruction in the conduit. PROCEDURE: Angiography showed a heavily calcified conduit with moderate insufficiency. During pre-dilation, a total of six balloons ruptured due to heavy conduit calcification prior to reaching desired inflation diameter. Subsequently, double balloon technique was attempted using two 9-mm Conquest balloons. One of the conquest balloons was then replaced by 16-mm Atlas balloon and conduit dilation was performed. At full inflation, the Conquest balloon ruptured. The deflated ruptured Conquest balloon was kept in the conduit and the Atlas balloon was exchanged for 18 and then 20-mm Atlas balloons and both were used to dilate the conduit. The deflated ruptured Conquest balloon protected the Atlas balloons and conduit angioplasty proceeded successfully without further rupture of any balloon. Subsequently, four stents were placed in the conduit followed by delivery of Melody Valve using a 20-mm Ensemble system with excellent results. CONCLUSION: This case illustrates a novel method of using the body of a ruptured balloon to protect subsequent balloons from rupture due to heavy conduit calcification. This method requires the presence of two venous access lines but might save time, effort, and cost from repeated balloon ruptures.

CRISP: Catheterization RISk score for Pediatrics: A Report from the Congenital Cardiac Interventional Study Consortium (CCISC).

OBJECTIVES: We sought to develop a scoring system that predicts the risk of serious adverse events (SAE's) for individual pediatric patients undergoing cardiac catheterization procedures. BACKGROUND: Systematic assessment of risk of SAE in pediatric catheterization can be challenging in view of a wide variation in procedure and patient complexity as well as rapidly evolving technology. METHODS: A 10 component scoring system was originally developed based on expert consensus and review of the existing literature. Data from an international multi-institutional catheterization registry (CCISC) between 2008 and 2013 were used to validate this scoring system. In addition we used multivariate methods to further refine the original risk score to improve its predictive power of SAE's. RESULTS: Univariate analysis confirmed the strong correlation of each of the 10 components of the original risk score with SAE attributed to a pediatric cardiac catheterization (P < 0.001 for all variables). Multivariate analysis resulted in a modified risk score (CRISP) that corresponds to an increase in value of area under a receiver operating characteristic curve (AUC) from 0.715 to 0.741. CONCLUSION: The CRISP score predicts risk of occurrence of an SAE for individual patients undergoing pediatric cardiac catheterization procedures.

Hemodynamic response to ketamine in children with pulmonary hypertension.

BACKGROUND: The safety of ketamine in children with pulmonary hypertension has been debated because of conflicting results of prior studies in which changes in mean pulmonary artery pressure (mPAP) and pulmonary vascular resistance (PVR) have been widely variable. The goal of this prospective study was to quantitate the effects of ketamine on pulmonary hemodynamics in a cohort of children with pulmonary hypertension under conditions in which variables such as airway/ventilatory management, FiO(2), and use of vasodilating anesthetics were controlled. METHODS: The IRB approved this study of 34 children undergoing cardiac catheterization for pulmonary hypertension studies. Following anesthetic induction with sevoflurane and tracheal intubation facilitated by the administration of rocuronium 0.7-1 mg·kg(-1) iv, sevoflurane was discontinued and anesthesia was maintained with midazolam 0.1 mg·kg(-1) iv (or 0.5 mg·kg(-1) po preoperatively) and remifentanil iv infusion 0.5-0.7 mcg·kg(-1) ·min(-1). Ventilation was mechanically controlled to maintain PaCO(2) 35-40 mmHg. When endtidal sevoflurane was 0% and FiO(2) was 0.21, baseline heart rate (HR), mean arterial pressure (MAP), mPAP, right atrial pressure (RAP), pulmonary artery occlusion pressure (PAOP), right ventricular end-diastolic pressure (RVEDP), cardiac output, and arterial blood gases were measured, and indexed systemic vascular resistance (SVRI), indexed pulmonary vascular resistance (PVRI), and cardiac index (CI) were calculated. Each child then received a bolus of ketamine 2 mg·kg(-1) infused over 2 min. Measurements and calculations were repeated 2 min after the conclusion of the infusion. RESULTS: The mean (95% CI) increase in mPAP following ketamine was 2 mmHg (0.2, 3.7), which was statistically significant but clinically insignificant. PVRI and PVRI/SVRI did not change significantly. Hemodynamic changes did not differ among subjects with differing severity of pulmonary hypertension or between subjects chronically treated with pulmonary vasodilators or not. CONCLUSION: Ketamine is associated with minimal, clinically insignificant hemodynamic changes in sedated, mechanically ventilated children with pulmonary hypertension.

Diagnostic Utility of Three-Dimensional Rotational Angiography in Congenital Cardiac Catheterization.

We evaluated the diagnostic utility of the three modalities of three-dimensional rotational angiography (3DRA): rotational angiography (RA), multiplanar reformat (MPR) and three-dimensional angiographic reconstruction (3D-R) in pediatric cardiac catheterization. The 3DRA studies were classified by anatomy of interest based on our injection protocol: pulmonary arteries (PA), aorta, cavopulmonary connection (CPC), and others. Retrospective review of 3DRA images by two reviewers for each modality was conducted with grading as inferior, similar, or superior in comparison with the diagnostic quality of fixed-plane angiography (FPA). The percentages of grades for each modality were averaged. Weighted kappa statistic was used to evaluate inter-rater reliability. In total, 114 3DRA studies were performed on 87 patients between August 2010 and March 2012. Median age was 2.7 years (1 day-48.4 years) and median weight 12.1 kg (3.6-106.5 kg). For RA: 79.4 % of the studies were of diagnostic quality and 52.2 % were superior; 3D-R: 82 % were of diagnostic quality and 65.8 % were superior; and MPR: 83.5 % were of diagnostic quality and 63 % were superior. Overall 3DRA technologies (RA, 3D-R, MPR) were of diagnostic quality or better in 111/114 (97.4 %) studies and 103/114 (90.4 %) were judged superior. Most common reasons for inferior grading were limited opacification and metallic artifact. In pediatric cardiac catheterization, 3DRA imaging was of diagnostic quality and frequently provided additional clinically relevant data when compared to FPA.

Use of rotational angiography in assessing relationship of the airway to vasculature during cardiac catheterization.

OBJECTIVES: We are the first to describe the use of three-dimensional rotational angiography (3DRA) in creating multiplanar reconstruction (MPR) and volume rendering, in the catheterization suite, of airways at risk for compression by adjacent cardiac structures. BACKGROUND: 3DRA has emerged as a promising tool for improved visualization of cardiac and vascular structures in congenital heart disease. METHODS: This is a retrospective review of all available cases at our institution in which MPR from 3DRA was used to assess airways in relation to surrounding cardiovascular structures. RESULTS: Eight cases were reviewed from January 1, 2011 to November 30, 2013. Seven children had complex biventricular anatomy, including repaired truncus arteriosus, repaired absent pulmonary valve, repaired double outlet right ventricle, and vascular rings. One child had double inlet left ventricle and had undergone a hybrid procedure (stenting of the patent ductus arteriosus and banding of bilateral pulmonary arteries) before the Glenn procedure. Six of these cases involved distortion or stenosis of the pulmonary conduit or branch pulmonary arteries. In all cases, the trachea and the main bronchi were clearly visualized using MPR. Management was affected by the visualization of the airways during the catheterization procedure in seven of eight cases. Four cases had intraprocedural bronchoscopy that confirmed airway findings seen by MPR. In one case, computed tomography confirmed left bronchial compression seen by MPR. CONCLUSIONS: 3DRA can visualize airway anatomy and its relationship to the vasculature accurately. This has significant implications for preinterventional planning, intraprocedural management, as well as postprocedural recovery.

SCAI expert consensus statement for advanced training programs in pediatric and congenital interventional cardiac catheterization.

Pediatric and Congenital Interventional Cardiology is the practice of catheter-based techniques that improve cardiac physiology and circulation through the treatment of heart disease in children and adults with congenital or acquired heart defects. Over the last decade, and since last published training guidelines for pediatric cardiac catheterization and interventional cardiology were published in 2005 [1] the field of Pediatric and Congenital Cardiac Catheterization has evolved into a predominantly interventional discipline. As there is no sub-specialty certification for interventional cardiac catheterization in pediatrics, the Congenital Heart Disease Committee of the Society of Cardiovascular Angiography and Interventions has put together this consensus statement for advanced training in pediatric and congenital interventional cardiac catheterization. The statement puts forth recommendations for program infrastructure in terms of teaching, personnel, equipment, facilities, conferences, patient volume and trainee assessment. This is meant to set a standard for training programs as well as giving applicants a basis on which to judge and compare programs.

Standardizing radiation dose reporting in the pediatric cardiac catheterization laboratory-a multicenter study by the CCISC (Congenital Cardiovascular Interventional Study Consortium).

OBJECTIVES: We examine normalized air Kerma area product (PKA ) by body weight (PKA /BW) as a reference value of radiation dose and benchmark PKA /BW in pediatric laboratories using a multicenter registry database. BACKGROUND: Reduction of radiation dose is an important quality improvement task in pediatric cardiac catheterization laboratories. Physicians need to agree on a standard method of reporting radiation dose that would allow comparisons to be made between operators and institutions. METHODS: This was a multicenter observational study of radiation dose in pediatric laboratories. Patient demographic, procedural and radiation data including fluoroscopic time and PKA (µGy m(2) ) were analyzed. PKA /BW was obtained by indexing PKA to body weight. RESULTS: A total of 8,267 pediatric catheterization procedures (age <18 years) were included from 16 institutions. The procedures consisted of diagnostic (n = 2,827), transplant right ventricular (RV) biopsy (n = 1,172), and interventional catheterizations (n = 4268). PKA correlated with body weight better than with age and best correlated with weight-fluoroscopic time product. PKA /BW showed consistent values across pediatric ages. Interventional catheterizations had the highest PKA /BW (50th, 75th, and 90th percentiles: 72, 151, and 281 µGy m(2) /kg), followed by diagnostic (59, 105, and 175 µGy m(2) /kg) and transplant RV biopsy (27, 79, and 114 µGy m(2) /kg). CONCLUSION: PKA /BW appeared to be the most reliable standard to report radiation dose across all procedure types and patient age. We recommend PKA /BW to be used as the standard unit in documenting radiation usage in pediatric laboratories and can be used to evaluate strategies to lower radiation dosage in pediatric patients undergoing cardiac catheterizations. © 2014 Wiley Periodicals, Inc.

Pulmonary Artery Stent Implantation.

Pulmonary artery stent implantation has become integral in the treatment of pulmonary artery stenosis and is probably the most efficacious therapy for these lesions. Advancements in technology involving stent design and the equipment used for stent delivery have made this procedure much safer and more effective. Strategies to mitigate and successfully treat adverse events related to pulmonary artery stent implantation are reasonably well-established. Pulmonary artery stent implantation remains one of the most complex and technically challenging of congenital cardiac interventions.

Percutaneous transhepatic access for catheter ablation of cardiac arrhythmias.

AIMS: Femoral venous access may be limited in certain patients undergoing electrophysiology (EP) study and ablation. The purpose of this study is to review a series of patients undergoing percutaneous transhepatic access to allow for ablation of cardiac arrhythmias. METHODS AND RESULTS: Six patients with a variety of cardiac arrhythmias and venous abnormalities underwent percutaneous transhepatic access. Under fluoroscopic and ultrasound guidance, a percutaneous needle was advanced into a hepatic vein and exchanged for a vascular sheath over a wire. Electrophysiology study and radiofrequency ablation was then performed. All tachycardias, including atrial tachycardia, atrial flutter, atrioventricular nodal tachycardia, and atrial fibrillation, were ablated. Procedural times ranged from 227 to 418 min. Fluoroscopy times ranged from 32 to 95 min. There were no complications. All six patients have been arrhythmia-free in follow-up (5-49 months, mean 23.1 months). CONCLUSION: Percutaneous transhepatic access is safe and feasible in patients with limited venous access who are undergoing EP study and ablation for a range of cardiac arrhythmias.

Beeman Phillips (1927-2023).

This article memorializes Beeman Phillips (1927-2023). In 1956, Phillips accepted a position in the Department of Educational Psychology at the University of Texas at Austin and developed its school psychology program, which he directed from 1965 to 1992. In 1971, the program became the first APA-accredited school psychology program in the country. He was an assistant professor (1956-1961), associate professor (1961-1968), full professor (1968-1998), and an emeritus professor in retirement. Beeman was among the early school psychologists from varied backgrounds who developed training programs and shaped the field's structure. His philosophy of school psychology was perhaps best expressed in his School Psychology at a Turning Point: Ensuring a Bright Future for the Profession (1990). (PsycInfo Database Record (c) 2023 APA, all rights reserved).

3-D guidance of complex pulmonary artery stent placement using reconstructed rotational angiography with live overlay.

Technologies which allow rotational angiographic image acquisition and three dimensional (3-D) vascular reconstruction with subsequent fluoroscopic registration for live 3-D image overlay are rapidly being adopted. We present the first use of rotational angiography with integrated 3-D image overlay for guidance during complex pulmonary artery stent placement. Technical considerations, advantages, and limitations of this procedural strategy are discussed.

Cryo-balloon angioplasty for pulmonary vein stenosis in pediatric patients.

This study sought to determine the safety and effectiveness of cryo-balloon angioplasty (CbA) for pulmonary vein stenosis (PVS) in pediatric patients. Current therapy options for PVS are less than satisfactory due to recurrent progressive restenosis and neointimal proliferation. Catheterization database, hospital records, imaging studies, and pathologic specimens were reviewed for procedural-related and outcomes data in all patients who underwent pulmonary vein (PV) CbA using the Boston Scientific PolarCath Peripheral Dilation System between August 2006 and June 2009. Thirteen patients (19 PVs; median age 13 months [range 3.5 months to 18.5 years] and weight 7.9 kg [range 3.8 to 47.7]) underwent CbA. Mean PVS diameter after CbA increased from 2.19 (± 0.6) to 3.77 (± 1.1) mm (p < 0.001). Mean gradient decreased from 14 (± 7.4) to 4.89 (± 3.2) mm Hg (p < 0.001). Mean stenosis-to-normal vein diameter ratio increased from 0.52 (± 0.15) to 0.89 (± 0.33) (p < 0.001). Eight patients underwent repeat catheterization a mean of 5.6 months (± 3.66) later. Improved PVS diameter was maintained in 2 PVs. Four veins had restenosis but maintained diameters greater than that before initial CbA. In 11 PVs, the diameter decreased from 4.28 (± 1.14) to 2.53 (± 0.9) mm (p = 0.001). Mean gradient increased from 3.55 (± 3.0) to 14.63 (± 9.6) mm Hg (p = 0.011). All vessels underwent repeat intervention with acute relief of PVS. Stroke occurred within 24 h of CbA in 1 patient. CbA of PVS is safe and results in acute relief of stenosis. However, CbA appears minimally effective as the sole therapy in maintaining long-term relief of PVS.

Clinical manifestations and long-term follow-up in pediatric patients living at altitude with isolated pulmonary artery of ductal origin.

This study's aim was to define the clinical manifestations and long-term outcome of pediatric patients living at altitude with isolated pulmonary artery (PA) of ductal origin (IPADO). This was a retrospective cohort study of 17 consecutive cases of IPADO at a single center. All patients lived at modest altitude (median 2050 m [range 1700 m to 3050 m]). Fifteen children (88%) were symptomatic at presentation. High-altitude pulmonary edema was present in 2 patients (12%) at diagnosis, and only 1 patient had episodes of hemoptysis during follow-up. Fourteen patients (82%) demonstrated evidence of pulmonary arterial hypertension (PAH). Among 14 patients with PAH, 11 patients had surgical interventions. PAH resolved in 5 of 11 patients (45%) undergoing surgical rehabilitation. One patient died during follow-up, and 7 patients are receiving oral vasodilator therapies due to residual PAH; 14 patients remained asymptomatic. Our study showed that early intervention in patients with IPADO at modest altitude can potentially rehabilitate the isolated PA and reverse PAH. Whether surgery is indicated for patients with this disorder in the absence of PAH is unknown.