Marked lateral ST-segment elevation with inferior ST-segment depression in an asymptomatic 12 year-old girl: A normal variant?

Significant ST-segment changes raise concern for myocardial ischemia, cardiomyopathy or myocardial inflammation and therefore, warrant an extensive and often invasive cardiovascular evaluation. We report a 12 year-old asymptomatic African-American girl with marked ST-segment elevation in leads I and aVL and ST-segment depression in inferior leads II, III and aVF. Extensive cardiovascular evaluation did not reveal any abnormality suggesting that these findings, which have previously not been reported, are likely benign, at least in this young girl.

Minimum Travel Distance Among Publicly Insured Infants with Severe Congenital Heart Disease: Potential Impact of In-state Restrictions.

Travel distance to surgical centers may be increased when coverage restrictions prevent children with congenital heart disease (CHD) from receiving care at out-of-state congenital heart surgery centers. We estimated the minimum travel distance to congenital heart surgery centers among publicly insured infants with time-sensitive CHD surgical needs, under two different scenarios: if they were and were not restricted to in-state centers. Using 2012 Medicaid Analytic eXtract data from 40 states, we identified 4598 infants with CHD that require surgery in the first year of life. We calculated the minimum travel distance between patients' homes and the nearest cardiac surgery center, assuming patients were and were not restricted to in-state centers. We used linear regression to identify demographic predictors of distance under both scenarios. When patients were not restricted to in-state centers, mean minimum travel distance was 43.7 miles, compared to 54.1 miles when they were restricted. For 5.9% of patients, the difference in travel distance under the two scenarios exceeded 50 miles. In six states, the difference in mean minimum travel distance exceeded 20 miles. Under both scenarios, distance was positively predicted by rural status, residence in middle-income zip codes, and white/non-Hispanic or American Indian/Alaskan Native race/ethnicity. For some publicly insured infants with severe CHD, facilitating the receipt of out-of-state care could mitigate access barriers. Existing efforts to regionalize care at fewer centers should be designed to avoid exacerbating access barriers among publicly insured CHD patients.

Serial Versus Direct Dilation of Small Diameter Stents Results in a More Predictable and Complete Intentional Transcatheter Stent Fracture: A PICES Bench Testing Study.

Balloon-expandable stents, implanted in infants and children with congenital heart disease (CHD), often require redilation to match somatic growth. Small diameter stents may eventually require longitudinal surgical transection to prevent iatrogenic vascular stenosis. Intentional transcatheter stent fracture (TSF) is an emerging alternative approach to stent transection, but little is known about the optimal stent substrate and best protocol to improve the likelihood of successful TSF. Bench testing was performed with a stent dilation protocol. After recording baseline characteristics, stents were serially or directly dilated using ultra-high-pressure balloons (UHPB) until fracture occurred or further stent dilation was not possible. Stent characteristics recorded were as follows: cell design, metallurgy, mechanism, and uniformity of fracture. Stents tested included bare-metal coronary stents, premounted small diameter stents, and ePTFE-covered small diameter premounted stents. Ninety-four stents representing 9 distinct models were maximally dilated, with 80 (85%) demonstrating evidence of fracture. Comprehensive fracture details were recorded in 64 stents: linear and complete in 34/64 stents (53.1%), linear and incomplete in 9/64 stents (14.1%), transverse/complex and complete in 6/64 stents (9.4%), and transverse/complex and incomplete in 15/64 stents (23.4%). Stent fracture was not accomplished in some stent models secondary to significant shortening, i.e., "napkin-ring" formation. Serial dilation resulted in evidence of fracture in 62/67 (92.5%) stents compared with 18/27 (66.7%) stents in the direct dilation group (p = 0.003). Intentional TSF is feasible in an ex vivo model. Serial dilation more reliably expanded the stent and allowed for ultimate stent fracture, whereas direct large diameter dilation of stents was more likely to generate a "napkin-ring" configuration, which may be more resistant to fracture. In vivo animal and human testing is necessary to better understand the response to attempted TSF for newly developed stents as well as those currently in use.

Transcatheter closure of patent ductus arteriosus using the AMPLATZER™ duct occluder II (ADO II).

OBJECTIVES: The study purpose is to evaluate the safety and efficacy of the ADO II device for closure of patent ductus arteriosus (PDA) in children. BACKGROUND: Transcatheter treatment of PDA has been evolving for 40+ years and is the treatment of choice. The AMPLATZER™ Duct Occluder (ADO) device was developed for larger diameter ducts and is not ideal in all PDAs. ADO II was developed for small to moderate-sized ducts. METHODS: This is a single-arm, multicenter study evaluating safety and efficacy of the ADO II device. Patients <18 years were screened for a PDA ≤5.5 mm in diameter and 3-12 mm in length. Right and left heart catheterization was performed, and hemodynamic data were obtained at the time of implant. The diameter of the left pulmonary artery (LPA) and descending aorta, and the presence of any pre-existing pressure gradients across the LPA or aortic arch were assessed at baseline and 6 months post-implant. RESULTS: A total of 192 patients were enrolled. The median implant time was 74 min. Median fluoroscopy time was 12 min. A retrograde (aortic) approach was used in 33% of procedures and demonstrated a statistically significant reduction in fluoroscopy time (P value = 0.0018) compared to an antegrade approach. The device was successfully implanted in 93% of patients, with complete closure in 98% of successful implantations. CONCLUSIONS: In this prospective study, the ADO II was safe and effective for closure of small to moderate PDAs. Implantation is simple and the ability for retrograde aortic delivery reduces procedure-related radiation exposure. © 2017 Wiley Periodicals, Inc.

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.

Endovascular stent provides more effective early relief of SVC obstruction compared to balloon angioplasty.

OBJECTIVE: To determine whether superior vena cava (SVC) stent implantation is superior to balloon angioplasty for relieving SVC stenosis. BACKGROUND: SVC stent and balloon dilation have been used as treatment for SVC stenosis. Although safe and effective, outcome data comparing the two methods are limited. METHODS: A Pediatric Cardiac Care Consortium review identified SVC stenosis. Patients who required SVC intervention were divided into two subgroups-balloon dilation (Group A) and stent implantation (Group B). Logistic regression and the log-rank test were used to test the need for re-intervention within 6 months after the initial procedure. RESULTS: SVC intervention was performed on 210/637 patients with SVC stenosis (33%). There were 108/210 (51%) patients with balloon dilation (Group A) and 102/210 (49%) with stent implantation (Group B). Re-intervention within 6 months of the initial intervention was more common in Group A compared to Group B [Group A = 31/40 (77.5%); Group B = 5/22 (22.7%)]. The odds-ratio for re-intervention within 6 months of the initial procedure for balloon vs. stent, is 7.3 [95% CI: (2.91, 22.3), P < 0.0001]. In addition, during the first 6 months after an intervention for SVC stenosis the proportion of patients with stent implantation that remained free of re-intervention was significantly higher than after balloon angioplasty (log-rank test, P < 0.0001). Neither age nor weight was significantly associated with the need for re-intervention. CONCLUSIONS: SVC stent implantation is more effective than angioplasty in relief of SVC obstruction. Weight and age are not risk factors for early re-intervention.

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.

Pericardial effusion after pediatric hematopoietic cell transplant.

PE can occur following HCT. However, the incidence, etiology, risk factors, and treatment remain unclear. We performed a retrospective study evaluating 355 pediatric recipients of HCT treated at a single institution between January 2005 and August 2010. No cases of PE were identified in the autologous HCT (auto-HCT) recipients (0/43), while 19% (57/296) of allogeneic HCT (allo-HCT) developed PE. Among the 57 PE patients, 40 (70%) were males; the median age at transplantation was 6.6 yr (0.1-17.3 yr). Thirty-six patients (63%) had significant PE with 23 patients (40%) treated by pericardiocentesis, and 19 (33%) experiencing recurrent PE. OS rates for patients who developed PE were 84% at 100 days and 65% at three yr after HCT. Risk factors associated with PE on multivariate analysis included myeloablative conditioning (p = 0.01), delayed neutrophil engraftment (p < 0.01), and CMV + serostatus of the recipient (p = 0.03). Recipients with non-malignant diseases were significantly less likely to die after development of PE (p = 0.02 and 0.004 when comparing with standard and high-risk diseases, respectively). In summary, PE is a common and significant complication of pediatric allo-HCT. Prospective studies are needed to better determine the etiology and optimal method of PE treatment after HCT.

Transcatheter closure of the perimembranous ventricular septal defect-preclinical trial of a new Amplatzer device.

OBJECTIVES: This study assessed the feasibility and efficacy of implanting a new nitinol device for closure of perimembranous ventricular septal defects in a swine model. BACKGROUND: Perimembranous ventricular septal defect occurs in 80% of patients requiring treatment for congenital heart disease. METHODS: The Amplatzer perimembranous ventricular septal occluder device (pmVSO2 device, AGA Medical Company, Plymouth MN) is a new transcatheter Nitinol device containing polyester fabric designed to close the perimembranous ventricular septal defect (VSD). The device has 75% reduction in radial force, 45% reduction in clamping force, and increased stability as compared to the previous version. The device was implanted in six swine with naturally occurring perimembranous VSD with immediate, 1, 7, ∼30, and ∼90 day followup by echocardiography, angiography, and final pathological examination. RESULTS: The device was successfully implanted in all animals and was retrievable and repositionable. There was complete occlusion of the VSD in five of six cases without embolization. There was no thrombus formation on the device or occurrence of complete heart block. A single instance of a tiny residual shunt was attributed to capture of tricuspid valve apparatus. CONCLUSIONS: The success of this animal study confirms safety and feasibility of the Amplatzer pmVSO2 device. Human trials are planned.

Stenting of aortic coarctation: acute, intermediate, and long-term results of a prospective multi-institutional registry--Congenital Cardiovascular Interventional Study Consortium (CCISC).

INTRODUCTION: Since the 1980s, stent implantation has evolved as an important therapeutic strategy for coarctation of the aorta. However, available data is frequently flawed by short follow-up, lack of adequate follow-up imaging, and retrospective nature of data collection. METHODS: Data was prospectively collected using a multicenter registry congenital cardiovascular interventional study consortium (CCISC). Between 2000 and 2009, 302 patients from 34 centers with a median weight of 58 kg underwent stent implantation for coarctation. Eligible patients (44%) completed intermediate follow-up (3-18 months) with integrated imaging (cath, CT, MRI), whereas 21% completed long-term follow-up (>18-60 months). Procedural success was defined as UL/LL systolic gradient of less than 20 mm Hg, lack of significant recurrent obstruction, and freedom from unplanned repeat intervention. RESULTS: Acute procedural success was 96%. Cumulative intermediate success was 86%, and cumulative long-term success was 77%. Unplanned repeat interventions were required in 4%, and aortic wall complications were seen in 1% of patients (dissection n = 1 and aneurysm n = 3). Other adverse events (n = 15) occurred mainly acutely and included technical complications such as stent malposition (n = 9). At long-term follow-up, 23% of patients continued to have systolic blood pressure above the 95th centile, 9% had an upper-to-lower limb blood pressure gradient in excess of 20 mm Hg, and 32% were taking antihypertensive medication. CONCLUSIONS: This study documented acute, intermediate, and long-term outcome data comparable or superior with other surgical or interventional series. However, even with successful initial stent therapy, patients continue to require long-term follow-up and have associated long-term morbidity, relating to aortic wall complications, systemic hypertension, recurrent obstruction as well as need for repeat intervention.

Experimental evaluation of a new articulated Amplatzer ductal occluder device without fabric.

OBJECTIVES: To describe a new percutaneous PDA device. BACKGROUND: The ADO II was developed by AGA Medical for closure of small-moderate sized PDAs via a small delivery catheter from an antegrade or retrograde catheter approach. The objective of this study was to evaluate the technical feasibility, safety, and efficacy of the ADO II in a canine PDA model. METHODS: The ADO-II consists of multi-layer nitinol wire braid with symmetric retention disks and an articulating connecting center waist, without sewn-in polyester, that can be delivered though a 4-5F catheter. A PDA was surgically created in nine dogs. Transcatheter occlusion of the PDA was performed using the ADO II. Angiographic and hemodynamic data were obtained at 7, 30, 60, and 90 days post-procedure. The devices were then harvested for pathology. RESULTS: Devices were placed transarterially (n = 8) and transvenously (n = 1). All PDAs were occluded and there were no significant pressure gradients (P > 0.05) at the immediate and 90 post-implant evaluations. Pathology found endothelial coverage on all aortic and pulmonary disks, except at the tip of the microscrew. There were no procedural complications. One canine was euthanized 4 hr after device implant because of a clinical deterioration. The staff veterinarian and pathologist concluded that the animal's illness was not device related. CONCLUSIONS: The Amplatzer ADO II devices can be safely deployed in animal models of PDAs, with complete resolution of the PDA shunt. The lower profile and symmetry of the ADO II allows for venous or arterial approach and smaller delivery catheter size. The ADO-II is expected to be a preferred alternative for closure of small-moderate PDAs.

Stent placement for palliation of cor triatriatum dexter in a dog with suspected patent foramen ovale.

An 11 month old spayed, female dog presented with exercise intolerance and cyanosis upon exertion. Echocardiography revealed an imperforate cor triatriatum dexter with mild tricuspid valve dysplasia, an underfilled right ventricle and significant right to left shunting across a presumptive patent foramen ovale. Balloon dilation of the abnormal atrial membrane was initially successful in creating a communication between the right atrial chambers, but stenosis of the original perforation and persistent clinical signs prompted a second intervention. A balloon expandable biliary stent was placed across the abnormal partition, improving caudal venous return to the right ventricle and reducing the right to left shunt. Three months after stent placement, resting oxygen saturation had normalized. Six months after stent placement, exercise tolerance had improved and exertional cyanosis had resolved. Long term follow up will be necessary to assess for remodeling of the right ventricle with improved venous return. Stent placement can be considered as a palliative treatment option for cor triatriatum dexter, especially for stenosis post-balloon dilation.

Expansion Characteristics of Stents Used in Congenital Heart Disease: Serial Dilation Offers Improved Expansion Potential Compared to Direct Dilation: Results from a Pediatric Interventional Cardiology Early Career Society (PICES) Investigation.

OBJECTIVE: Intravascular stents are now routinely used to treat young patients with vascular stenoses. Future stent redilations are often necessary to account for somatic growth. The purpose of this study was to compile a database of characteristics for stents commonly used in the treatment of congenital heart disease patients, and compare serial dilation to direct dilation to the maximal diameter. DESIGN: A standardized bench testing protocol was established and utilized in the assessment of all stents. Ultra high pressure balloons were used to serially dilate each stent by set increments until the stent reached at least 24 mm in diameter, developed a napkin-ring configuration, or fractured. Length and diameter of each stent were measured at baseline and following each stage of dilation. Maximal stent diameters, foreshortening properties, and ability to fracture were reported. Stents were then tested for direct dilation from the primary diameter to the maximal diameter, and the same data was obtained. RESULTS: A total of 127 stents were bench-tested, 80 of which were serially dilated and 47 directly dilated. Most premounted stents could be serially dilated to approximately twice their stated nominal diameter. All tested unmounted stents could be serially dilated to ≥20 mm. Foreshortening occurred at larger diameters, but varied significantly among different stent types. Serial dilation offered more consistent results with significantly less foreshortening and more symmetric expansion when compared with direct dilation. Most premounted stents could be fractured when serially dilated. CONCLUSIONS: All tested vascular stents can be dilated beyond their nominal implantation diameter. Serial dilation offers a much more reliable response with uniform expansion, less foreshortening, greater maximal diameter, and improved intentional fracture potential, as compared to direct dilation from the nominal to maximal diameter. In vivo studies are necessary to corroborate these findings in the congenital heart disease population.

Palliative balloon angioplasty in a cat with right pulmonary arterial branch stenoses and concurrent absence of the left pulmonary artery.

Unilateral absence of the left or right pulmonary artery with concurrent contralateral pulmonary arterial branch stenoses is an exceptionally rare disorder. We describe this anomaly in a cat with severe exercise intolerance and respiratory distress. Transthoracic echocardiography and angiography demonstrated the absence of the left pulmonary artery with concurrent right pulmonary branch stenosis. Palliative balloon angioplasty of the right pulmonary artery substantially reduced right ventricular systolic pressure load and alleviated the patient's clinical signs.

Frequency of superior vena cava obstruction in pediatric heart transplant recipients and its relation to previous superior cavopulmonary anastomosis.

The risk factors for superior vena cava (SVC) obstruction after pediatric orthotopic heart transplantation (OHT) have not been identified. This study tested the hypothesis that pretransplant superior cavopulmonary anastomosis (CPA) predisposes patients to SVC obstruction. A retrospective review of the Pediatric Cardiac Care Consortium registry from 1982 through 2007 was performed. Previous CPA, other cardiac surgeries, gender, age at transplantation, and weight at transplantation were assessed for the risk of developing SVC obstruction. Death, subsequent OHT, or reoperation involving the SVC were treated as competing risks. Of the 894 pediatric OHT patients identified, 3.1% (n = 28) developed SVC obstruction during median follow-up of 1.0 year (range: 0 to 19.5 years). Among patients who developed SVC obstruction, 32% (n = 9) had pretransplant CPA. SVC surgery before OHT was associated with posttransplant development of SVC obstruction (p <0.001) after adjustment for gender, age, and weight at OHT and year of OHT. Patients with previous CPA had increased risk for SVC obstruction compared with patients with no history of previous cardiac surgery (hazard ratio 10.6, 95% confidence interval: 3.5 to 31.7) and to patients with history of non-CPA cardiac surgery (hazard ratio 4.7, 95% confidence interval: 1.8 to 12.5). In conclusion, previous CPA is a significant risk factor for the development of post-heart transplant SVC obstruction.

Comparison of surgical, stent, and balloon angioplasty treatment of native coarctation of the aorta: an observational study by the CCISC (Congenital Cardiovascular Interventional Study Consortium).

OBJECTIVES: The purpose of this study was to compare the safety and efficacy of surgical, stent, and balloon angioplasty (BA) treatment of native coarctation acutely and at follow-up. BACKGROUND: Controversy surrounds the optimal treatment for native coarctation of the aorta. This is the first multicenter study evaluating acute and follow-up outcomes of these 3 treatment options in children weighing >10 kg. METHODS: This is a multicenter observational study. Baseline, acute, short-term (3 to 18 months), and intermediate (>18 months) follow-up hemodynamic, imaging data, and complications were recorded. RESULTS: Between June 2002 and July 2009, 350 patients from 36 institutions were enrolled: 217 underwent stent, 61 underwent BA, and 72 underwent surgery. All 3 arms showed significant improvement acutely and at follow-up in resting systolic blood pressure and upper to lower extremity systolic blood pressure gradient (ULG). Stent was superior to BA in achieving lower ULG acutely. Surgery and stent were superior to BA at short-term follow-up in achieving lower ULG. Stent patients had shorter hospitalization than surgical patients (2.4 vs. 6.4 days; p < 0.001) and fewer complications than surgical and BA patients (2.3%, 8.1%, and 9.8%; p < 0.001). The BA patients were more likely to encounter aortic wall injury, both acutely and at follow-up (p < 0.001). CONCLUSIONS: Stent patients had significantly lower acute complications compared with surgery patients or BA patients, although they were more likely to require a planned reintervention. At short-term and intermediate follow-up, stent and surgical patients achieved superior hemodynamic and integrated aortic arch imaging outcomes compared with BA patients. Because of the nonrandomized nature of this study, these results should be interpreted with caution.

Successful Coil Embolization of a Large Ascending Aortic Pseudoaneurysm Following Explantation of the EXCOR Pediatric Ventricular Assist Device in a Patient With Acute Fulminant Myocarditis.

Mechanical ventricular assistance has become a reliable tool for the support of children and infants with heart failure. The devices have shown efficacy both as a bridge to transplantation and as a bridge to recovery. The potential complications that may occur with long-term support have not been fully described. This article reports the occurrence of a large pseudoaneurysm associated with the ascending aorta following explantation of the EXCOR Pediatric ventricular assist device. A management strategy for this potentially lethal complication is described.

Percutaneous stent placement in children weighing less than 10 kilograms.

The objectives of this study were to evaluate the safety, efficacy, and outcomes of palliative percutaneous stent placement in infants with congenital heart disease (CHD). There is interest in improving outcomes of infants with CHD through interventional/surgical collaboration. Small, high-risk patients may benefit from delayed open operations, and endovascular stents may provide a means to defer surgery to more advantageous times in select infants. Patients weighing < or =10 kg in whom stent placement was attempted during 2003-2006 were identified. Diagnoses, indications, angiographic and hemodynamic data, complications, and outcomes were reviewed. Seventeen stents were successfully placed in 15 patients. The mean age was 10.21 months; the mean weight was 6.08 kg. Indications were branch pulmonary artery stenosis, coarctation, total anomalous pulmonary venous return with obstructed venous egress, right ventricle-pulmonary artery conduit, and shunt stenosis. Premounted Palmaz Genesis and Driver Mx stents were used. Average vessel diameter increased from 3.65 to 6.72 mm (p < 0.001). The pressure gradient fell from 24 to 15 mm Hg (p < 0.01). Two stents migrated. One was recaptured and implanted at the target site and one implanted away from the target site. None needed surgical intervention. Mean follow-up was 270 days. Five patients have had additional surgery; seven patients underwent 10 additional stent dilations. There was no procedural-related mortality. Five patients (33%) died during the follow-up period, none attributable to the stent placement. We conclude that stent placement can be successfully accomplished in select, small, high-risk patients. A collaborative interventional/surgical paradigm is important to ensure that the risk-benefit ratio is properly evaluated during the patient selection.

Ductal stent and cavo-atrial sac occlusion in an adult with profound cyanosis after palliated cyanotic congenital heart disease.

We report a unique combination of PDA stent placement and occlusion of a persistent cavo-atrial connection in an adult with complex cyanotic congenital heart disease. The unusual anatomy and physiology with prior palliative surgery were amenable to catheterization intervention and have resulted in marked clinical improvement.

Pulmonary venous wedge pressure provides an accurate assessment of pulmonary artery pressure in children with a bidirectional Glenn shunt.

PURPOSE: In circulations with pulsatile pulmonary artery flow the pulmonary venous wedge pressure (PVWp) has been validated as a good estimate of pulmonary artery pressure (PAp), when PAp is low. The purpose of this study was to validate PVWp estimates of PAp in the less-pulsatile pulmonary circulation of children after bidirectional Glenn shunts. METHODS: A retrospective study was performed of 22 simultaneous measurements of PVWp and PAp made during 20 catheterizations in 19 children who had undergone bidirectional Glenn procedures. The PAp was measured directly from the branch PA ipsilateral to the side of the PVWp, or in the SVC. Pulmonary resistance (Rp) was calculated with both PAp and PVWp, to assess the impact of PAp estimates on Rp determinations. RESULTS: Patients ranged in age from 5 months to 10.7 years. There were a variety of univentricular cardiac malformations in the study group. Two children had antegrade pulmonary blood flow in addition to a bidirectional Glenn shunt. The mean PAp ranged from 4 to 14 mmHg, while mean PVWp ranged from 3 to 15 mmHg. Mean PVWp never differed from mean PAp by more than 3 mmHg. There was a significant linear relation between mean PAp and PVWp: PAp = 0.86 (PVWp) + 2.0 (R2 = 0.89; P < 0.0001). PVWp provided a good approximation of PAp regardless of the presence (n = 2) or absence (n = 19) of antegrade pulmonary flow. There was a good linear correlation between the Rp calculated by both methods (RpPAp = 0.9 (RpVWp) + 0.5; R2 = 0.74; P < 0.0001). CONCLUSION: The mean PVWp provides a close approximation of mean PAp in children with a bidirectional Glenn shunt and provides valuable hemodynamic information in cases where direct PAp measurements are unavailable.