Management of Pediatric Pulmonary Hypertension Using Remote Pulmonary Artery Pressure Sensor Monitoring

Background Management of pediatric pulmonary hypertension (PH) may require manipulation of multiple receptor sites to maximize response to medical therapy. Assessment of response typically occurs through imaging, labs, physical exam and recurrent cardiac catheterization, with anesthetic exposure to assess pulmonary artery pressures (PAP) and vascular resistance (PVR). We aimed to assess feasibility, safety and utility of remote PAP monitoring in pediatric PH patients. Methods We reviewed 4 pediatric patients with significant PH, each of whom underwent cardiac catheterization with pulmonary vasoreactivity testing and placement of a CardioMEMS remote PAP monitoring device. Results Four patients (P1-4: ages 5, 6, 8 and 10 years old) underwent CardioMEMS insertion without procedural complication. P1, P2 and P3 presented with unrepaired VSD;ASD with partial anomalous pulmonary venous return;and ASD and PDA, respectively, while P4 had prior repair of atrioventricular canal. Three patients had Down syndrome. All had elevated PAP and PVR. Mean left lower PA branch size was 7 mm. Mean PAP prior to therapy was 70 mm Hg for P1, 82 for P2, 93 for P3 and 30 for P4. All 4 patients required initiation of triple therapy for treatment of PH, with improvement or normalization of PAP by CardioMEMS, which also included surgical or catheter based intervention for 3 patients. Post-repair of P2, he was unable to be separated from cardiopulmonary bypass and was placed on ECMO. Right ventricular cardiac output improved over 2 weeks, with improvement of PAP determined through serial CardioMEMS. He was successfully decannulated, utilizing CardioMEMS in the OR. Two patients also developed COVID respiratory infections at home with CardioMEMS assessments allowing for oxygen and medication titration. Conclusion Remote PAP monitoring is feasible and appears safe in pediatric patients with adequate PA size. It allows for manipulation of medical therapy with real time knowledge of impact on PAP and can augment management during weaning of mechanical cardiac support. It may also augment decision-making in management of PH patients with developmental disabilities in whom traditional assessments may be more challenging.Copyright © 2023 American College of Cardiology Foundation

MANIFESTATIONS OF SCIMITAR SYNDROME IN AN ADULT

SESSION TITLE: Anatomical Cardiovascular Disease Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Scimitar Syndrome is a rare congenital disorder characterized by partial or complete anomalous pulmonary venous drainage to the inferior vena cava, right lung hypoplasia and displacement of the cardiac structures into the right hemi-thorax (1) The name Scimitar Syndrome is derived from the resemblance of the anomalous pulmonary vein to a curved Middle Eastern sword known as a scimitar (1). Some patients experience no symptoms while others may have severe pulmonary hypertension and dyspnea (2) (3). Cases may be discovered in infancy although some patients are not diagnosed until early adulthood. Here we present a case of a woman who was discovered to have symptomatic scimitar syndrome in her teenage years and opted to forego correction. CASE PRESENTATION: Patient was a 38 year old female with past medical history of Scimitar Syndrome who presented to the pulmonary clinic after a short hospitalization with mild COVID-19. Prior to contracting COVID-19 she had experienced worsening dyspnea over one year and now becomes short of breath after walking one half block. Open surgical correction was offered at diagnosis but was not pursued due to patient preference. A CT Angiogram of the chest was performed while she was hospitalized which showed an anomalous right pulmonary vein draining to the hepatic IVC, minimal bilateral patchy opacities and displacement of the heart into the right hemi-thorax. Echocardiography showed normal right ventricular size and function and did not show evidence of pulmonary hypertension. She was referred to the adult congenital heart disease clinic and continued to have severe dyspnea and fatigue. 6 months later, repeat echocardiography and cardiac MRI demonstrated borderline dilation of the right ventricle and atrium but no echocardiographic evidence of pulmonary hypertension. The patient remains hesitant to undergo invasive procedures or interventions. She has been offered a right heart catheterization to better characterize her volume status and obtain direct measurement of her pulmonary artery pressures. DISCUSSION: Although generally discovered in infancy and childhood, Scimitar syndrome may not be discovered until adulthood. Various treatments are available for correction of scimitar syndrome including open surgical approaches with direct implantation of the scimitar vein into the left atrium, trans-catheter occlusion of aorto-pulmonary collaterals and re-routing of the anomalous vein into the left atrium via endoscopic graft placement (4) (6) (7) (8). If left uncorrected worsening left to right shunting and pulmonary hypertension may occur (5). Our patient has developed borderline right ventricular dilation and has experienced severe functional limitation. A right heart catheterization is indicated to determine her pulmonary artery pressures. This case illustrates the potential consequences of deferring early treatment for Scimitar Syndrome CONCLUSIONS:. Reference #1: Frydrychowicz A, Landgraf B, Wieben O, François CJ. Images in Cardiovascular Medicine. Scimitar syndrome: added value by isotropic flow-sensitive four-dimensional magnetic resonance imaging with PC-VIPR (phase-contrast vastly undersampled isotropic projection reconstruction). Circulation. 2010 Jun 15;121(23):e434-6. doi: 10.1161/CIRCULATIONAHA.109.931857. PMID: 20547935 Reference #2: Abdullah A. Alghamdi, Mansour Al-Mutairi, Fahad Alhabshan, Scimitar syndrome: restoration of native pulmonary venous connection, European Heart Journal Supplements, Volume 16, Issue suppl_B, November 2014, Pages B41–B43, https://doi.org/10.1093/eurheartj/suu025 Reference #3: Khan A, Ring NJ, Hughes PD. Scimitar syndrome (congenital pulmonary venolobar syndrome). Postgrad Med J. 2005 Apr;81(954):216. doi: 10.1136/pgmj.2004.027813. PMID: 15811882;PMCID: PMC1743239 DISCLOSURES: No relevant relationships by John Prudenti No relevant relationships by Anthony Smith

Detrimental Intrauterine Stress Exposure and Post-natal Outcomes in Prenatally Diagnosed Congenital Heart Disease

Background: Prenatal diagnosis of congenital heart disease (CHD) is a stressful event that leads to anxiety, depression and traumatic stress in expectant parents. Cortisol is elevated in times of stress, and when present in mothers, crosses the placenta and leads to suppression of the fetus' own production of cortisol. When those neonates go on to require stressful procedures in the first months of life, some are unable to effectively mount a cortisol mediated stress response which can lead to poor outcomes and even death. We sought to investigate the relationship between maternal stress during pregnancy, and neonatal outcomes. Methods: We conducted a retrospective chart review of pregnancies complicated by a fetal diagnosis of critical CHD (including transposition of the great arteries, tetralogy of Fallot, total anomalous pulmonary venous return, and coarctation) who were born between 5/1/2019 and 5/1/2021. Maternal data included demographics and medical comorbidities. Composite maternal prenatal stress score (PSS) was calculated based upon 1) prenatal mental health diagnoses, 2) housing/food insecurity 3) income insecurity, 4) social support/child care, 5) legal involvement, 6) transportation issues and 7) other stressors. Categories ranged from 0 (no concerns) to 3 (significant concerns). Infant charts were reviewed for postnatal, and post-operative outcomes including infection, inotropic support, and exogenous steroid treatment. Results: 41 maternal-fetal dyads met inclusion criteria. Demographic and catheter based intervention at a median of 8 (2-54) days of life. 13 patients had single ventricle anatomy (8 initial surgical palliation, 5 catheterization). Mothers with higher composite PSS were more likely to have infants that required steroids after CHD surgery compared to mothers with lower scores (p=.01) (figure 1). Surgical patients needing bypass were more likely to require post-operative steroids than those not requiring bypass (18/22 vs 0/4, p<.005). None of the catheter-based interventions (including those with high risk single ventricle anatomy) required steroids (p <.0001). Maternal individual stress sub-categories, severity of prenatal CHD diagnosis, and counseling during the COVID-19 era did not correlate with steroid treatment. Finally, PSS did not correlate with individual outcomes such as birthweight, inotropic support, infection or hypoglycemia. Conclusion: Maternal prenatal stress is multifactorial and higher composite maternal prenatal stress scores are correlated with post-bypass steroid requirements, suggesting that a stressful intrauterine environment can be associated with worse postoperative outcomes for the neonate.

Bronchial carcinoid with bronchocele masquerading as Scimitar syndrome on chest radiograph.

Bronchial carcinoid tumors occur predominantly in the perihilar location and can be asymptomatic. They may present in early stages with only obstructive features such as mucus plugging of airways resulting in bronchocele formation. We report the case of a 44-year lady with no chest complaints, who underwent chest radiograph for a routine preanesthetic checkup. A vertically oriented, tubular, soft tissue density lesion was observed in the right lower lung, which mimicked a Scimitar vein. Scimitar syndrome is a congenital disorder in which an anomalous vein drains the middle and lower lobes of right lung and enters into the IVC most commonly. It may present asymptomatically in adults and on chest radiograph appears as a vertical tubular opacity paralleling the right cardiac border. However, CT angiography revealed the lung lesion to be a bronchocele, distal to a central intensely enhancing spherical mass, completely occluding the right lower lobe bronchus. This perihilar mass had been missed on the chest radiograph. Bronchoscopic biopsy revealed a carcinoid tumor. As the patient was asymptomatic, she refused surgery in the ongoing COVID-19 pandemic.