Body mass index and age are associated with ventricular end-diastolic pressure in adults with a Fontan circulation.

INTRODUCTION: Systemic ventricular end-diastolic pressure is an important haemodynamic variable in adult patients with Fontan circulation. Risk factors associated with elevated end-diastolic pressure have not been clearly identified in this population. METHODS: All patients > 18 years with Fontan circulation who underwent cardiac catheterisation at our centre between 1/08 and 3/19 were included. Relevant patient variables were extracted. Univariate and multivariate general linear models were analysed to identify variables associated with end-diastolic pressure. RESULTS: Forty-two patients were included. Median age was 24.0 years (20.9-29.0) with a body mass index of 23.7 kg/m2 (21.5-29.7). 10 (23.8%) patients had a systemic right ventricle. The median (Interquartile range) and mean pulmonary artery pressure were 11.0 mmHg (9.0-12.0) and 16.0 mmHg (13.0-18.0), respectively. On univariate analysis, end-diastolic pressure was positively associated with body mass index (p < 0.01), age > 25 years (p = 0.04), symptoms of heart failure (p < 0.01), systemic ventricular systolic pressure (p = 0.03), pulmonary artery mean pressure (p < 0.01), and taking diuretics (p < 0.01) or sildenafil (p < 0.01). End-diastolic pressure was negatively associated with aortic saturation (p < 0.01). On multivariate analysis, end-diastolic pressure was positively associated with age ≥ 25 years (p < 0.01), and body mass index (p = 0.04). CONCLUSIONS: In a cohort of adult patients with Fontan circulation undergoing catheterisation, end-diastolic pressure was positively associated with age ≥ 25 years and body mass index on multivariate analysis. Maintaining a healthy body mass index may offer haemodynamic benefit in adults with Fontan physiology.

Identifying key components of a web-based long term care planning intervention for family caregivers of individuals with intellectual/developmental disabilities.

BACKGROUND: Few family caregivers of individuals with intellectual or developmental disabilities develop long-term care (LTC) plans for their relative. Web-based interventions promoting LTC planning have potential for widespread adoption into clinical practice. METHODS: We conducted focus groups with 49 primary caregivers of individuals with intellectual or developmental disabilities in NY, PA, OH, DE, and TX to identify barriers and facilitators of LTC planning, review existing tools, and identify critical features for web-based LTC planning interventions. Participants also answered questions on demographic characteristics and functional status. RESULTS: NVivo qualitative analysis software was used to analyse focus groups using a grounded theory approach. Caregivers identified web tool accessibility and topics such as finances, housing, and government benefits as critical. Caregivers also described desired features for a LTC planning tool. CONCLUSIONS: This study identified desired characteristics of web-based LTC planning tools and ways in which existing web-based interventions might be adapted or enhanced.

Ductal stent endocarditis resulting in a large aortic pseudoaneurysm.

An infant with ductal dependent pulmonary blood flow who underwent neonatal ductal stenting and, 4 months later, developed ductal stent endocarditis due to Streptococcus gallolyticus subsp. pasteurianus was described. The infection was associated with a moderate aortic pseudoaneurysm and the patient was treated with antibiotics as well as surgical aortic pseudoaneurysm repair. This novel and unusual complication of ductal stent placement warrants reporting.

Identification of patient variables that are associated with ventricular end-diastolic pressure before the bidirectional Glenn operation.

INTRODUCTION: Systemic ventricular end-diastolic pressure is important in patients with single ventricle heart disease. Predictors of an elevated systemic ventricular end-diastolic pressure prior to bidirectional Glenn operation have been incompletely identified. METHODS: All patients who underwent bidirectional Glenn operation operation at our centre between January 2007 and March 2017 were retrospectively identified and patient variables were extracted. For patients who had undergone Fontan operation at the time of this study, post-Fontan patient variables were also extracted. RESULTS: One-hundred patients were included with a median age at pre-bidirectional Glenn operation catheterisation of 4.5 months. In total, 71 (71%) patients had a systemic right ventricle. At the pre-bidirectional Glenn operation catheterisation, the mean systemic ventricular end-diastolic pressure was higher amongst those with systemic right ventricle compared to left ventricle (9.1 mmHg ± 2.1 versus 7.7 ± 2.7 mmHg, p < 0.01). On univariate analysis, pre-bidirectional Glenn operation systemic ventricular end-diastolic pressure was positively associated with the presence of a systemic right ventricle (p < 0.01), history of recoarctation (p = 0.03), history of Norwood operation (p = 0.04), and ventricular systolic pressure (p < 0.01). On multivariate analysis, systemic ventricular end-diastolic pressure was positively associated with the presence of a systemic right ventricle (p < 0.01) and ventricular systolic pressure (p < 0.01). Amongst those who had undergone Fontan operation at the time of study (n = 49), those with a higher pre-bidirectional Glenn operation systemic ventricular end-diastolic pressure were more likely to have experienced death, transplantation, or listed for transplantation (p = 0.02) and more likely to have had heart failure symptoms (p = 0.04) at a mean time from Fontan of 5.2 years ± 1.3. CONCLUSIONS: In patients undergoing bidirectional Glenn operation operation, the volume-loaded, pre-bidirectional Glenn operation state may expose diastolic dysfunction that has prognostic value.

An unusual case of coronary artery compression that did not preclude successful transcatheter pulmonary valve placement.

During transcatheter pulmonary valve placement, coronary compression observed during simultaneous right ventricular outflow tract angioplasty and coronary angiography typically contraindicates valve implantation. We present a unique patient with tetralogy of Fallot who underwent successful transcatheter Melody valve placement despite coronary compression observed during right ventricular outflow tract balloon angioplasty.

Risk Factors for an Elevated Ventricular End-Diastolic Pressure Prior to the Fontan Operation.

Systemic ventricular end-diastolic pressure (SVEDP) is an important determinant of pulmonary artery pressure in those with a Fontan circulation. Predictors of an elevated SVEDP have been incompletely identified in this population. All who underwent the Fontan operation at our center between 1/2009 and 12/2013 were retrospectively identified. SVEDP at the pre-Fontan catheterization and other patient variables were extracted. We identified 61 patients. Pre-Fontan SVEDP was positively associated with systemic ventricular systolic pressure (ß = 0.4, p = 0.004), aortic systolic pressure (ß = 0.3, p = 0.007), aortic mean pressure (ß = 0.3, p = 0.02), and decreased ventricular systolic function (p = 0.03). Compared to those with pre-Fontan SVEDP ≤ 7 mmHg, patients with SVEDP > 7 mmHg had higher average ventricular systolic pressure (85.0 ± 7.5 vs. 78.7 ± 8.3 mmHg, p = 0.003), higher average descending aorta mean pressure (62.4 ± 4.9 vs. 58.6 ± 8.1 mmHg, p = 0.03), and a higher incidence of decreased ventricular systolic function (36 vs. 15%, p = 0.07). For those with a systemic right ventricle, the SVEDP decreased significantly from the pre-Stage 2 to pre-Fontan measurements (8.7 ± 2.6 vs. 7.3 ± 2.0 mmHg, p = 0.02), but not for those with a systemic left ventricle (7.8 ± 2.0 vs. 7.2 ± 1.8 mmHg, p = 0.3). At pre-Fontan catheterization, decreased ventricular systolic function and markers of systemic afterload were positively associated with the SVEDP. SVEDP decreased significantly after Stage 2 for those with a systemic right ventricle, but not for those with a systemic left ventricle; the systemic right ventricle may be particularly vulnerable to pre-Stage 2 volume loading.

Bone-Specific Alkaline Phosphatase in Patients Who Have Undergone the Fontan Operation.

Bone-specific alkaline phosphatase (BALP) is produced by osteoblasts. A recent series noted a positive association between cardiac index (CI) and BALP in patients with Fontan circulation. CI is low at baseline in these patients, and small decreases in CI may result in diverting of blood away from bone. We prospectively enrolled 15 patients (males ≤ 14 yo, females ≤ 12 yo) who had previously undergone Fontan operation and were undergoing cardiac catheterization. Serum BALP was measured at catheterization, and analysis performed to evaluate association between age-/gender-specific BALP z-score and CI as well as other patient variables. The median age at catheterization was 5.6 years (3.1-13.1), and time from Fontan was 1.5 years (0.1-12.1). The median superior vena cava saturation (SVC) was 65 % (52-74), median average between SVC and inferior vena cava (IVC) saturations was 62.5 % (51-70), and median CI was 3.8 L/min/m(2) (2.0-8.4). The median BALP was 65 IU/L and BALP z-score was -2.1 (-3.2 to 0.9). BALP z-score was not associated with CI (ρ = -0.1, p = 0.7), but a positive correlation was noted with the average of SVC and IVC saturation (ρ = 0.5, p = 0.052) and with SVC saturation (ρ = 0.4, p = 0.07), both nearly reaching statistical significance. In our cohort of children with Fontan circulation undergoing catheterization, BALP z-score was not associated with CI, but an association with estimates of mixed venous saturation was noted that nearly reached statistical significance. We hypothesize that BALP is a marker of oxygen delivery in those with Fontan circulation and may represent a valuable biomarker in this population.

Outcomes and predictors of reintervention in patients with pulmonary atresia and intact ventricular septum treated with radiofrequency perforation and balloon pulmonary valvuloplasty.

Radiofrequency perforation and valvuloplasty (RFV) is an effective initial treatment in patients with pulmonary atresia and intact ventricular septum (PA-IVS) and mild to moderate right ventricle and tricuspid valve hypoplasia. Outcomes and risk factors for the need for additional interventions in these patients are poorly defined. All patients with PA-IVS who underwent RFV at our center between January 2000 and July 2011 were reviewed. Twenty-three patients met the inclusion criteria. All patients underwent successful valvuloplasty with no procedural deaths and one major complication. Excluding two patients with limited follow-up, 6 (29 %) patients underwent no subsequent interventions, whereas 9 (42 %) patients underwent surgical right-ventricular outflow tract augmentation. All except one patient with adequate follow-up have a biventricular circulation with saturation >92 %. Patients who did not undergo any right-ventricular outflow tract intervention after valvuloplasty had a significantly lower gradient across the pulmonary valve after valvuloplasty (9.9 mmHg ± 8.4 vs. 19.1 mmHg ± 10.4, p = 0.05). Significantly more patients who received a supplemental source of pulmonary blood flow had a tricuspid valve z-score <-0.7 compared with patients who did not receive supplemental blood flow [2 (15 %) vs. 7 (70 %), p = 0.008]. In our cohort of patients with PA-IVS, radiofrequency perforation with valvuloplasty was an effective and safe first step in establishing a biventricular circulation. Postvalvuloplasty pulmonary valve gradient may be predictive of subsequent outflow tract intervention, and tricuspid hypoplasia may be predictive of the need for a supplemental source of pulmonary blood flow.

Percutaneous closure of patent ductus arteriosus in small infants with significant lung disease may offer faster recovery of respiratory function when compared to surgical ligation.

OBJECTIVES: To describe our experience with percutaneous closure of patent ductus arteriosus (PDA) in small infants and compare outcomes to matched surgical patients. BACKGROUND: Ligation via thoracotomy has been used to close PDAs in small infants, but has been associated with respiratory and hemodynamic compromise. We hypothesized that percutaneous closure would offer faster recovery of respiratory function. METHODS: Patients <4 kg requiring positive pressure ventilation who underwent percutaneous PDA closure between January 2000 and April 2012 were reviewed and matched to contemporary surgical patients on gestational age (GA), birth weight (BW), procedure weight (WT), and ventilation mode. Patients returned to baseline respiratory status when the product of mean airway pressure and FiO2 returned to pre-procedural levels. RESULTS: Eight matched pairs were included. Median BW, GA, and WT were 1.43 kg (0.52-2.97), 29.8 weeks (24-39), and 2.8 kg (2.2-3.9) for catheter patients and 1.55 kg (0.48-3.04), 29 weeks (23-37), and 2.75 kg (2.3-4.2) for surgical patients. Complete PDA closure occurred in all. The median time to return to baseline respiratory status was significantly shorter in the percutaneous group (17 hr (range 0-113) vs. 53 hr (range 13-219), P < 0.05). In the percutaneous group, two patients developed mild aortic coarctation, one mild left pulmonary artery stenosis, and four femoral vascular thromboses which all resolved with medical therapy. Surgical complications included significant respiratory and cardiac compromise, rib fractures and urinary retention. CONCLUSIONS: Percutaneous closure of PDA in small infants on respiratory support is equivalent in safety and efficacy and may offer shorter recovery time than surgical ligation.

Portal and sinusoidal fibrosis are common on liver biopsy after Fontan surgery.

Hepatic fibrosis is an important complication after Fontan surgery in patients with single-ventricle congenital heart disease. Few reports of hepatic histology in these patients exist, and sinusoidal fibrosis has been described. We aimed to characterize fibrosis at liver biopsy procedure in patients with previous Fontan surgery and to identify patient variables associated with the degree of fibrosis. All patients who had previous Fontan surgery and who subsequently underwent liver biopsy at our institution between January 1990 and July 2010 were identified. For each biopsy specimen, portal and sinusoidal fibrosis were graded and medical records reviewed. Biopsy specimens from 13 patients were examined; the median time from Fontan surgery to liver biopsy procedure was 16.9 years (range 6.9-25). At the most recent biopsy procedure, 12 patients (92 %) had evidence of portal fibrosis, including 1 patient with portal-based cirrhosis. Thirteen patients (100 %) had at least some degree of sinusoidal fibrosis, including 1 patient with centrilobular-based cirrhosis. Lower platelet count was associated with greater degree of portal fibrosis by ordinal regression (odds ratio 0.84, P = 0.04), and patients with no or mild portal fibrosis had significantly higher platelet counts compared with those with moderate or severe portal disease (278 ± 78 K vs. 160 ± 46 K, P = 0.005). Four patients underwent serial biopsy procedures; portal fibrosis was progressed in 3 patients, and sinusoidal fibrosis was progressed in 3 patients. After Fontan surgery, portal and sinusoidal fibrosis are common at liver biopsy and can progress over time. Lower platelet count may represent a marker of portal-based disease in these patients.

Chest pain with elevated troponin assay in adolescents.

OBJECTIVE: We sought to describe the evaluation, treatment, and follow-up of adolescents who presented to a single institution with chest pain and an elevated troponin I value in the absence of typical symptoms of pericarditis or myocarditis. Materials and methods We performed a retrospective review of patients in the age group of 10-18 years of age with no history of significant heart disease admitted to our institution from 2000 to 2010 after presenting with chest pain and an elevated troponin I value. RESULTS: A total of 16 patients were identified with a median age of 16.5 years (range 11.2-17.8 years). Of these 13 (81%) were male and 10 (63%) showed evidence of localised ST elevations on electrocardiogram. The median peak troponin I level was 17.8 nanograms per millilitre (range 0.89-227, normal less than 0.4). There were eight patients (50%) with a diagnosis of coronary vasospasm, three patients (20%) with atypical myopericarditis, one patient with coronary anomaly, one patient with hypercoagulable disorder, and one patient with prolonged supraventricular tachycardia. In two patients, no definitive diagnosis was made. There was one patient who needed catheter-based intervention, which involved stenting of a coronary artery after a procedure-related complication. CONCLUSIONS: In our cohort of adolescents without history of significant cardiac disease, chest pain and elevated troponin I levels were attributed to a variety of causes. Although coronary vasospasm and atypical myopericarditis were seen most commonly, coronary anomaly was identified in one case. Magnetic resonance imaging proved a useful diagnostic tool to assess coronary artery anatomy and myocardial changes suggestive of myocarditis. On the basis of these results and a review of the literature, a general evaluation algorithm is presented.

The relationship between left ventricular dilation and right ventricular diastolic function in children with a patent ductus arteriosus.

The impact of a dilated left ventricular (LV) on right ventricular (RV) diastolic function has not been investigated. We hypothesized that in patients with a patent ductus arteriosus (PDA), LV dilation causes elevation of the RV end-diastolic pressure (RVEDP) through ventricular-ventricular interaction. We identified patients' ages 6 months to 18 years who underwent transcatheter PDA closure at our center from 2010 to 2019. One hundred and thirteen patients were included with a median age of 3 years (0.5-18). The median LV end-diastolic dimension (LVEDD) Z-score was 1.6 (-1.4-6.3). RVEDP was positively associated with RV systolic pressure (0.38, P < 0.01), ratio of pulmonary artery/aortic systolic pressure (0.4, P < 0.01), and pulmonary capillary wedge pressure (0.71, P < 0.01). RVEDP was not associated with LVEDD Z-score (0.03, P = 0.74). In children with a PDA, RVEDP was not associated with LV dilation, but was positively associated with RV systolic pressure.

Changes in left ventricular systolic function after transcatheter patent ductus arteriosus closure in premature infants.

Background: Changes in left ventricular (LV) systolic function have not been well described in premature neonates after transcatheter patent ductus arteriosus (PDA) closure. Methods and Results: We retrospectively identified all premature neonates < 3 kg who underwent a transcatheter PDA closure at our center between January 1 2015 and January 31, 2021. LV indices before and after closure were extracted and an analysis was performed. Overall, 23 neonates were included with a mean procedural weight of 1894 ± 622 g. At 24 h after closure, the median left ventricular ejection fraction (LVEF) (66% interquartile range [IQR] 12% vs. 61% IQR 12, P< 0.001) and median LV end-diastolic dimension z-score (3.3 IQR 1.8 vs. 1.4 IQR 2.6, P < 0.001) both decreased and 5 (22%) neonates had an LVEF <55%. Patients who had an LVEF <55% at 24 h had a higher preprocedure LV end-diastolic dimension z-score (4.2 IQR 1.2 vs. 2.8 IQR 1.6, P = 0.01), a higher preprocedure LV end-diastolic volume (19 mL IQR 4 mL vs. 11 mL IQR 11, P = 0.03), a higher birth weight (940 g IQR 100 g vs. 760 g IQR 140, P = 0.04), and were more likely to receive intravenous calcium during the procedure (60% vs. 11%, P = 0.04) compared to those with an LVEF ≥55% at 24 h after closure. Of those with LVEF <55% at 24 h, all normalized before discharge. Conclusion: In preterm neonates who underwent successful transcatheter PDA closure, 23% developed abnormal LVEF after closure and those with significant LV dilation before the procedure were at increased risk for the development of LVEF <55% after closure.

A Novel Case of Marfan Syndrome in an Infant With Hypoplastic Left Heart Syndrome.

This report describes a case of hypoplastic left heart syndrome (HLHS) and Marfan syndrome presenting in conjunction, and highlights how a connective tissue disorder may alter medical and surgical management of newborns with HLHS.

Surgical Palliation of Right Aortic Arch With an Isolated Innominate Artery From the Left Ductus Arteriosus.

A right aortic arch with an isolated left innominate artery from the left patent ductus arteriosus is a rare arch anomaly, and establishing continuity between the innominate artery and aorta can be challenging. We describe repair of this lesion in a three-week-old male using an autologous pedicle flap of ascending aorta as well as a homograft patch as the roof to recreate continuity between the aorta and left innominate artery.

Superior Vena Cava Banding to Facilitate Unilateral Bidirectional Glenn Operation in Patients With Single Ventricle Heart Disease and Bilateral Superior Caval Veins.

Staged palliation to achieve a total cavopulmonary connection is a common treatment strategy in patients with single ventricle congenital heart disease. Patients with bilateral superior caval veins (bilateral SVC) often require the creation of bilateral superior cavopulmonary connections as part of the staged palliation, and these patients are at increased risk of morbidity. We describe a novel technique used in two patients with bilateral SVC and very small (1-2 mm) bridging vein that encouraged bridging vein growth and facilitated creation of a unilateral superior cavopulmonary connection.

Treatment of Severe Native Left Pulmonary Artery Stenosis With Coronary Stent Implantation in a 2.4 kg Neonate.

A 1-month-old, 2.4 kg infant, previously born at 32 weeks gestation, was found to have a murmur while in the neonatal intensive care unit. The patient had ongoing feeding intolerance and required supplemental oxygen via nasal cannula. Cardiac computed tomography showed discrete stenosis of the proximal left pulmonary artery (LPA) with a normal-sized distal LPA. We describe the treatment course with transcatheter coronary stent implantation.

Lymphopenia in Patients with Single-ventricle Heart Disease after the Fontan Operation.

BACKGROUND: Lymphopenia is common in patients with Fontan circulation and no history of protein-losing enteropathy, but this phenomenon has not been significantly described in the literature. METHODS: We retrospectively identified patients with Fontan circulation who underwent catheterization between January 2003 and January 2013 at our center. Patients who had complete blood count with differential drawn within 12 months of the catheterization were included. Patients were excluded if complete blood count with differential was drawn in setting of possible infection or if there was history of protein-losing enteropathy (PLE). Possible associations between patient characteristics and absolute lymphocyte count (ALC) were examined. RESULTS: Fifteen patients were included. The median age at catheterization was 10.2 years (3.8-26.9) and median time from Fontan operation was 6.5 years (0.7-22.1). Twelve (80%) patients had undergone extracardiac Fontan and 9 (60%) had fenestration placed. The median time between complete blood count with differential and catheterization was 2 days (0-346). The median inferior vena cava (IVC) pressure was 13 mm Hg (7-20). The median ALC was 1.5 × 10(3) /µL (0.8-4.5). Four patients (26.7%) met criteria for lymphopenia with ALC < lower limit of normal and 7 (46.7%) patients had an ALC ≤ lower limit of normal. ALC was not associated with any hemodynamic variables but was associated with platelet count (rho = 0.5, P = .04), total white blood cell count (rho = 0.8, P ≤ .001), and absolute monocyte count (0.7, P = .002). CONCLUSIONS: In a cohort of patients with Fontan circulation and no history of protein-losing enteropathy who underwent catheterization, lymphopenia was common and positively associated with low platelet count. Thrombocytopenia has been shown to correlate with the degree of hepatic fibrosis in those with Fontan and, thus, hepatic fibrosis may underlie lymphopenia in these patients.

Hybrid Pulmonary Artery Stenting at the Bidirectional Glenn or Fontan Operation in Patients With Single Ventricle Congenital Heart Disease.

BACKGROUND: Stenosis of the retroaortic pulmonary artery is common in patients with single ventricle heart disease. Intraoperative hybrid stenting at the bidirectional Glenn or Fontan operation can treat this lesion and avoid a complex surgical arterioplasty. METHODS: Patients who underwent intraoperative stent implantation to the retroaortic pulmonary artery during the bidirectional Glenn or Fontan operation at our center between January 2005 and July 2014 were retrospectively identified. RESULTS: Thirteen patients were included with a median weight of 8 kg (5.6-14.4 kg) and age of 6 months (4 months-3.8 years). All had undergone Norwood operation, and eight (62%) had hypoplastic left heart syndrome. Eight (62%) underwent stent placement during bidirectional Glenn and five (38%) during Fontan operation. Ten patients had one stent placed, and three had two overlapping stents. The median diameter of the stenotic vessel was 3 mm (2.0-5.5 mm) and diameter of the balloon used for stent expansion was 7 mm (5-10 mm). Two complications occurred including pulmonary hemorrhage from presumed wire perforation and left main stem bronchus compression requiring stent removal. No patient required stent intervention in the postoperative period, and all were discharged from the hospital. At a median follow-up of 1.3 years (2 months-7.1 years), six patients underwent interval dilation to account for somatic growth. CONCLUSIONS: Hybrid stenting of the retroaortic pulmonary artery at the bidirectional Glenn or Fontan operation is an effective treatment of pulmonary artery stenosis and prevents the need for a complex surgical arterioplasty.