Joshua Murphy

New Definition and Natural History of Patients With Diffuse Pulmonary Arteriovenous Malformations: Twenty-Seven–Year Experience

BACKGROUND Patients with diffuse pulmonary arteriovenous malformations (PAVM), a small but important subset of the PAVM population, have significant morbidity and mortality rates. METHODS Thirty-six patients (21 female and 15 male) with diffuse PAVM from a cohort of 821 consecutive patients with PAVM were evaluated. Diffuse PAVM were categorized angiographically: involvement of one or more segmental pulmonary arteries in one or both lungs. Hereditary hemorrhagic telangiectasia (HHT) status, gender, presence or absence of large (> or = 3-mm diameter artery) focal PAVM, oxygen saturations, complications including hemoptysis, years of follow-up, and survival were tabulated. RESULTS HHT was present in 29 of 36 patients (81%), and diffuse PAVM were more commonly bilateral (26 of 36 patients, 72%) than unilateral (10 of 36 patients, 28%) [p = 0.02]. Female gender was associated with bilateral diffuse PAVM (19 of 26 patients, 73%) [p = 0.01]. Focal PAVM were present in both groups but more commonly in patients with bilateral involvement (16 of 26 patients, 62%) [p = 0.02]. Initial oxygen saturations (pulse oximetry, standing) of patients with unilateral and bilateral diffuse PAVM were 87 +/- 7% and 79 +/- 8% (mean +/- SD), respectively (p = 0.02). The last or current values for patients with unilateral and bilateral involvement are 95 +/- 3% and 85 +/- 7%, respectively (p < 0.0001). Nine deaths occurred, and all were in patients with bilateral involvement. Deaths were due to hemoptysis of bronchial artery origin (n = 2), hemorrhage from duodenal ulcer (n = 1), spontaneous liver necrosis (n = 3), brain hemorrhage (n = 1), brain abscess (n = 1), and operative death during attempted lung transplant (n = 1). CONCLUSIONS Patients with diffuse PAVM are a high-risk group, and yearly follow-up is recommended.

ORIGINAL RESEARCHPULMONARY VASCULAR DISEASENew Definition and Natural History of Patients With Diffuse Pulmonary Arteriovenous Malformations: Twenty-Seven–Year Experience

BACKGROUND Patients with diffuse pulmonary arteriovenous malformations (PAVM), a small but important subset of the PAVM population, have significant morbidity and mortality rates. METHODS Thirty-six patients (21 female and 15 male) with diffuse PAVM from a cohort of 821 consecutive patients with PAVM were evaluated. Diffuse PAVM were categorized angiographically: involvement of one or more segmental pulmonary arteries in one or both lungs. Hereditary hemorrhagic telangiectasia (HHT) status, gender, presence or absence of large (> or = 3-mm diameter artery) focal PAVM, oxygen saturations, complications including hemoptysis, years of follow-up, and survival were tabulated. RESULTS HHT was present in 29 of 36 patients (81%), and diffuse PAVM were more commonly bilateral (26 of 36 patients, 72%) than unilateral (10 of 36 patients, 28%) [p = 0.02]. Female gender was associated with bilateral diffuse PAVM (19 of 26 patients, 73%) [p = 0.01]. Focal PAVM were present in both groups but more commonly in patients with bilateral involvement (16 of 26 patients, 62%) [p = 0.02]. Initial oxygen saturations (pulse oximetry, standing) of patients with unilateral and bilateral diffuse PAVM were 87 +/- 7% and 79 +/- 8% (mean +/- SD), respectively (p = 0.02). The last or current values for patients with unilateral and bilateral involvement are 95 +/- 3% and 85 +/- 7%, respectively (p < 0.0001). Nine deaths occurred, and all were in patients with bilateral involvement. Deaths were due to hemoptysis of bronchial artery origin (n = 2), hemorrhage from duodenal ulcer (n = 1), spontaneous liver necrosis (n = 3), brain hemorrhage (n = 1), brain abscess (n = 1), and operative death during attempted lung transplant (n = 1). CONCLUSIONS Patients with diffuse PAVM are a high-risk group, and yearly follow-up is recommended.

Balloon Angioplasty and Stenting of Branch Pulmonary Arteries Adverse Events and Procedural Characteristics: Results of a Multi-Institutional Registry

Background— Pulmonary artery (PA) balloon angioplasty and/or stenting (PA rehabilitation) is one of the most common procedures performed in the cardiac catheterization laboratory, but comprehensive and consistently reported data on procedure-related adverse events (AE) are scarce. Methods and Results— Data were prospectively collected using a multicenter registry (Congenital Cardiac Catheterization Project on Outcomes). All cases that included balloon angioplasty and/or stent implantation in a proximal or lobar PA position were included. Multivariate analysis was used to evaluate for independent predictors of AE and need for early reintervention. Between February 2007 and December 2009, 8 institutions submitted details on 1315 procedures with a PA intervention. An AE was documented in 22% with a high severity (level 3 to 5) AE in 10% of cases. Types of AE included vascular/cardiac trauma (19%), technical AE (15%), arrhythmias (15%), hemodynamic AE (14%), bleeding via endotracheal tube/reperfusion injury (12%), and other AE (24%). AE were classified as not preventable in 50%, possibly preventable in 41%, and preventable in 9%. By multivariate analysis, independent risk factors for level 3 to 5 AE were presence of ≥2 indicators of hemodynamic vulnerability, age below 1 month, use of cutting balloons, and operator experience of <10 years. Reintervention during the study period occurred in 22% of patients undergoing PA rehabilitation. Conclusions— PA rehabilitation is associated with a 10% incidence of high-level severity AE. Hemodynamic vulnerability, young age, use of cutting balloons, and lower operator experience were significant independent risk factors for procedure-related AE.Background— Pulmonary artery (PA) balloon angioplasty and/or stenting (PA rehabilitation) is one of the most common procedures performed in the cardiac catheterization laboratory, but comprehensive and consistently reported data on procedure-related adverse events (AE) are scarce. Methods and Results— Data were prospectively collected using a multicenter registry (Congenital Cardiac Catheterization Project on Outcomes). All cases that included balloon angioplasty and/or stent implantation in a proximal or lobar PA position were included. Multivariate analysis was used to evaluate for independent predictors of AE and need for early reintervention. Between February 2007 and December 2009, 8 institutions submitted details on 1315 procedures with a PA intervention. An AE was documented in 22% with a high severity (level 3 to 5) AE in 10% of cases. Types of AE included vascular/cardiac trauma (19%), technical AE (15%), arrhythmias (15%), hemodynamic AE (14%), bleeding via endotracheal tube/reperfusion injury (12%), and other AE (24%). AE were classified as not preventable in 50%, possibly preventable in 41%, and preventable in 9%. By multivariate analysis, independent risk factors for level 3 to 5 AE were presence of ≥2 indicators of hemodynamic vulnerability, age below 1 month, use of cutting balloons, and operator experience of <10 years. Reintervention during the study period occurred in 22% of patients undergoing PA rehabilitation. Conclusions— PA rehabilitation is associated with a 10% incidence of high-level severity AE. Hemodynamic vulnerability, young age, use of cutting balloons, and lower operator experience were significant independent risk factors for procedure-related AE.

Balloon Angioplasty and Stenting of Branch Pulmonary Arteries

Background— Pulmonary artery (PA) balloon angioplasty and/or stenting (PA rehabilitation) is one of the most common procedures performed in the cardiac catheterization laboratory, but comprehensive and consistently reported data on procedure-related adverse events (AE) are scarce. Methods and Results— Data were prospectively collected using a multicenter registry (Congenital Cardiac Catheterization Project on Outcomes). All cases that included balloon angioplasty and/or stent implantation in a proximal or lobar PA position were included. Multivariate analysis was used to evaluate for independent predictors of AE and need for early reintervention. Between February 2007 and December 2009, 8 institutions submitted details on 1315 procedures with a PA intervention. An AE was documented in 22% with a high severity (level 3 to 5) AE in 10% of cases. Types of AE included vascular/cardiac trauma (19%), technical AE (15%), arrhythmias (15%), hemodynamic AE (14%), bleeding via endotracheal tube/reperfusion injury (12%), and other AE (24%). AE were classified as not preventable in 50%, possibly preventable in 41%, and preventable in 9%. By multivariate analysis, independent risk factors for level 3 to 5 AE were presence of ≥2 indicators of hemodynamic vulnerability, age below 1 month, use of cutting balloons, and operator experience of <10 years. Reintervention during the study period occurred in 22% of patients undergoing PA rehabilitation. Conclusions— PA rehabilitation is associated with a 10% incidence of high-level severity AE. Hemodynamic vulnerability, young age, use of cutting balloons, and lower operator experience were significant independent risk factors for procedure-related AE.Background— Pulmonary artery (PA) balloon angioplasty and/or stenting (PA rehabilitation) is one of the most common procedures performed in the cardiac catheterization laboratory, but comprehensive and consistently reported data on procedure-related adverse events (AE) are scarce. Methods and Results— Data were prospectively collected using a multicenter registry (Congenital Cardiac Catheterization Project on Outcomes). All cases that included balloon angioplasty and/or stent implantation in a proximal or lobar PA position were included. Multivariate analysis was used to evaluate for independent predictors of AE and need for early reintervention. Between February 2007 and December 2009, 8 institutions submitted details on 1315 procedures with a PA intervention. An AE was documented in 22% with a high severity (level 3 to 5) AE in 10% of cases. Types of AE included vascular/cardiac trauma (19%), technical AE (15%), arrhythmias (15%), hemodynamic AE (14%), bleeding via endotracheal tube/reperfusion injury (12%), and other AE (24%). AE were classified as not preventable in 50%, possibly preventable in 41%, and preventable in 9%. By multivariate analysis, independent risk factors for level 3 to 5 AE were presence of ≥2 indicators of hemodynamic vulnerability, age below 1 month, use of cutting balloons, and operator experience of <10 years. Reintervention during the study period occurred in 22% of patients undergoing PA rehabilitation. Conclusions— PA rehabilitation is associated with a 10% incidence of high-level severity AE. Hemodynamic vulnerability, young age, use of cutting balloons, and lower operator experience were significant independent risk factors for procedure-related AE.

Safety of Percutaneous Patent Ductus Arteriosus Closure: An Unselected Multicenter Population Experience

Background The technique and safety of transcatheter patent ductus arteriosus (PDA) closure have evolved during the past 20 years. We sought to report a multicenter experience of PDA closure with a focus on the rate of adverse events (AE) and a review of institutional practice differences. Methods and Results Outcome data on transcatheter PDA closure were collected at 8 centers prospectively using a multicenter registry (Congenital Cardiac Catheterization Project on Outcome Registry). Between February 2007 and June 2010, 496 PDA closures were recorded using a device in 338 (68%) or coils in 158 (32%). Most patients had an isolated PDA (90%). Fifty percent of patients were between 6 months and 3 years old, with only 40 patients (8%) <6 months old. Median minimum PDA diameter was 2.5 mm (range 1 to 12 mm; IQR 2 to 3 mm) for device closure and 1 mm (range 0.5 to 6 mm; IQR 1 to 2 mm) for coil closure (P<0.001). A device rather than coil was used in patients <3 years, weight <11 kg, and with a PDA minimum diameter >2 mm (all P<0.001). Three of 8 centers exclusively used a device for PDAs with a diameter >1.5 mm. In 9% of cases (n=46), an AE occurred; however, only 11 (2%) were classified as high severity. Younger age was associated with a higher AE rate. Coil‐related AEs were more common than device‐related AEs (10% versus 2%, P<0.001). Conclusions PDA closure in the present era has a very low rate of complications, although these are higher in younger children. Technical intervention‐related events were more common in coil procedures compared with device procedures. For PDAs ≤2.5 mm in diameter, institutional differences in preference for device versus coil exist.

Improved survival after heart transplant for failed Fontan patients with preserved ventricular function.

BACKGROUND Patients with a failing Fontan continue to have decreased survival after heart transplant (HT), particularly those with preserved ventricular function (PVF) compared with impaired ventricular function (IVF). In this study we evaluated the effect of institutional changes on post-HT outcomes. METHODS Data were retrospectively collected for all Fontan patients who underwent HT. Mode of failure was defined by the last echocardiogram before HT, with mild or no dysfunction considered PVF and moderate or severe considered IVF. Outcomes were compared between early era (EE, 1995 to 2008) and current era (CE, 2009 to 2014). Management changes in the CE included volume load reduction with aortopulmonary collateral (APC) embolization, advanced cardiothoracic imaging, higher goal donor/recipient weight ratio and aggressive monitoring for post-HT vasoplegia. RESULTS A total of 47 patients were included: 27 in the EE (13 PVF, 14 IVF) and 20 in the CE (12 PVF, 8 IVF). Groups were similar pre-HT, except for more PLE in PVF patients. More patients underwent APC embolization in the CE (80% vs 28%, p < 0.01). There was no difference in donor/recipient weight ratio between eras. There was a trend toward higher primary graft failure for PVF in the EE (77% vs 36%, p = 0.05) but not the CE (42% vs 75%, p = 0.20). Overall, 1-year survival improved in the CE (90%) from the EE (63%) (p = 0.05), mainly due to increased survival for PVF (82 vs 38%, p = 0.04). CONCLUSIONS Post-HT survival for failing Fontan patients has improved, particularly for PVF. In the CE, our Fontan patients had a 1-year post-HT survival similar to other indications.

Balloon valvuloplasty for congenital aortic stenosis: Multi-center safety and efficacy outcome assessment.

To describe contemporary outcomes of balloon aortic valvuloplasty (BAVP) performed in 22 US centers. Background: BAVP constitutes first‐line therapy for congenital aortic stenosis (cAS) in many centers.

Practice Variation in Single-Ventricle Patients Undergoing Elective Cardiac Catheterization: A Report from the Congenital Cardiac Catheterization Project on Outcomes (C3PO).

OBJECTIVE The objective of this study was to investigate variation in practice surrounding elective cardiac catheterization in patients with single-ventricle (SV) congenital heart disease. DESIGN Patient and procedural characteristics and outcomes during SV catheterization were collected prospectively from eight centers using a web-based registry (Congenital Cardiac Catheterization Project on Outcomes). We attempted to identify a population of elective procedures by limiting the cohort in case type and timing. Cases were then stratified by stage of SV palliation (pre-bidirectional cavopulmonary anastomosis [pre-BCPA], pre-Fontan and post-Fontan) and limited by age. Subcohort analysis was performed by mode of airway management (assisted vs. spontaneous ventilation). Institutional variation was assessed. RESULTS Between 2/2007 and 6/2010, 1459 (10.1%) of 14 467 cases in the registry met the inclusion and exclusion criteria, including 326 pre-BCPA, 571 pre-Fontan and 562 post-Fontan procedures. Median patient age was 0.4 (interquartile range 0.3, 0.5), 2.6 (1.0, 3.4) and 9.6 (5.2, 15.4) years and weight was 5.6 (4.8, 6.4), 12.2 (10.5, 14), and 26.3 (16.6, 51.8) kg in the pre-BCPA, pre-Fontan and post-Fontan cohorts, respectively. Cases were more commonly diagnostic in the pre-BCPA cohort (57%) whereas they were more commonly interventional in the pre-Fontan (69%) and post-Fontan (77%) cohorts. At least one adverse event (AE) occurred in 210 cases (14.4%) overall, including 20% of pre-BCPA, 11% of pre-Fontan and 14% of post-Fontan catheterizations. Mode of airway management was associated with statistically significant, but clinically small differences in hemodynamic measures in the pre- and post-Fontan cohorts, but not in the pre-BCPA group. Considerable practice variation exists across centers with variability in airway management, AE rate, case type, interventions performed and fluoroscopy time, in all SV cohorts. CONCLUSIONS Elective catheterization in SV patients, frequently performed with intervention, is common among US congenital cardiac centers. However, important variation in practice exists during these procedures in SV patients, at all stages of palliation.

Percutaneous carotid artery access in infants < 3 months of age

Carotid artery access in infants with congenital heart disease undergoing cardiac catheterization via a surgical cut down has been well described. There is a paucity of information regarding percutaneous carotid artery (CA) access in infants <3 months.

Routine Cardiac Catheterization Prior to Fontan Operation: Is It a Necessity?

Prior to the Fontan procedure, patients with single ventricle physiology with Glenn shunt are typically referred for cardiac catheterization to assess hemodynamics and potentially provide interventional measures. Currently, echocardiography provides detailed information which together with other non-invasive imaging such as CT scan and MRI may obviate the need for routine cardiac catheterization prior to the Fontan procedure. In this study, we examine the findings in cardiac catheterization in this population to determine: (a) the accuracy of echocardiography in providing adequate information prior to the Fontan procedure, particularly in identifying those in need of per-catheter intervention, and (b) the percentage of patients requiring interventional procedures during cardiac catheterization. We performed a retrospective chart review of echocardiographic and cardiac catheterization data for patients who underwent pre-Fontan cardiac catheterization at our center in the period from 02/01/2008 to 02/28/2017. We aimed to re-examine the necessity of routine cardiac catheterization in all single ventricle patients. This was performed through examining pre-catheterization echocardiography reports and comparing them to findings of the subsequent cardiac catheterization reports. Echocardiography reports were evaluated for accuracy in identifying significant anatomical or hemodynamic findings, which may impact success of Fontan procedure as well as the ability of echocardiography to predict findings important to know prior to the Fontan procedure. In this cohort of 40 children, 3 patients were found to have significant hemodynamic findings through cardiac catheterization which were not previously known by echocardiography. In addition, 28 out of 40 patients (70%) required interventional procedures to address significant abnormalities (systemic to pulmonary arterial collaterals, pulmonary artery stenosis, aortic arch stenosis, etc.). All cases of aortic arch stenosis were detected by echocardiography, however, all patients who required systemic to pulmonary arterial or left SVC embolization were not detected by echocardiography. Furthermore, echocardiography did not detect the need for branch pulmonary artery stenosis in 50% of cases. Cardiac catheterization appears to be an essential part of patient assessment prior to Fontan completion in patients with single ventricle physiology. This current practice may change in the future if a non-invasive screening tool is found to have high positive and negative predictive values in identifying the subset of patients who require potential intervention in pre-Fontan cardiac catheterization.