Neonatal and Pediatric Pulmonary Vascular Disease

Transcription

1 Neonatal and Pediatric Pulmonary Vascular Disease Emma Olson, MS, ARNP Pediatric Cardiology Nurse Practitioner Canadian Respiratory Conference April 14, 2018

2 Financial Interest Disclosure (over the past 24 months) I have no conflict of interest

3 Learning Objectives Recognize clinical presentation and understand diagnostic work-up of pediatric PH Describe common pediatric etiologies of PH Describe new recommendations for screening and diagnostic work-up for patients with BPD

4 What is Pulmonary Vascular Disease? Hemodynamic Disease: Pulmonary Hypertension Mean pulmonary artery pressure of 25 mmhg at rest Pulmonary Vascular Resistance (PVR) >3 Structural Disease Abnormal lung vasculature Functional Disease Impaired constriction Impaired relaxation

5 PVD Pathophysiology Excessive pulmonary vasoconstriction Endothelial dysfunction (endothelial cells are the orchestrators of vascular pathology) Chronically impaired production of vasodilators (nitric oxide and prostacyclin) Prolonged overexpression of vasoconstrictors (endothelin-1)

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7 Clinical Presentation & Symptoms Early Symptoms: Dyspnea Fatigue Exercise Intolerance Late Symptoms: Cyanosis with exertion Chest pain Presyncope / Syncope Dyspnea at rest Symptoms in Infants: Failure to thrive Decreased activity Diaphoresis irritability

8 Signs/Exam Relevant History Prominent RV impulse Loud S2 PI/TR murmur JVD Hepatomegaly Edema Prematurity Pulmonary disease Family history Autoimmune disease Liver disease Travel Meds/toxins

9 Steven H. Abman et al. Circulation. 2015;132: Copyright American Heart Association, Inc. All rights reserved.

10 Echocardiogram Preliminary diagnosis of PH is made by echo Essential in any child with suspicion of PH Presence or absence of structural heart disease

11 Echocardiogram Estimated right ventricular pressure Tricuspid Regurgitation (TR) or early Pulmonary Insufficiency (PI) velocity Septal position RV size/hypertrophy/function Left heart disease causing increased LA pressure Shunt lesions and direction of flow VSD, PDA, ASD Velocity and flow direction

12 Champion et al (2009). Circulation. 120:

13 Cardiac Catheterization: Gold standard for diagnosis and response to therapy Right and left heart cath Saturations (r/o systemic-to-pulm shunts) Pressures Hemodynamics (CI, PVR, Qp:Qs) Reactivity testing + if fall in mpap and PVRi by 20% with unchanged or increased cardiac output Angiography AHA/ATS recommends cardiac cath prior to initiation of any PAH-targeted therapy

14 Important other tests Chest CT with angiography r/o thromboembolic disease Evaluate lung parenchyma Pulmonary venous return Exercise testing 6MWT CPET on bike or treadmill Sleep study r/o sleep apnea Establish need for supplemental O2 at night Swallow study r/o aspiration Genetic testing

15 WHO Classifications (Nice 2013) 1. Pulmonary arterial hypertension (PAH) Idiopathic, Heritable, Drug/toxin induced, Associated (connective tissue disease, HIV, portal hypertension, CHD), Pulmonary Venous Occlusive Disease, Persistent PH of the newborn 2. PH due to left heart disease LV systolic dysfunction, LV diastolic dysfunction, valvular disease, congenital/aquired left heart inflow/outflow tract obstruction, congenital cardiomyopathies 3. PH due to lung disease and/or hypoxia COPD, interstitial lung disease, other pulm diseases with mixed restrictive and obstructive pattern, sleepdisordered breathing, alveolar hypoventilation disorders, chronic exposure to high altitude, developmental lung diseases 4. Chronic thromboembolic PH 5. PH with unclear multifactorial mechanisms Hematologic disorders, chronic hemolytic anemia, myeloproliferative disorders, splenectomy, systemic disorders: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis, metabolic disorders: glycogen storage disease, Gaucher disase, thyroid disorders, Other: tumoral obstruction, fibrosing mediastinitis, chronic renal failure, segmental PH

16 Challenges with adult classifications Del Cerro et al, PVRI Pediatric Taskforce, Panama 2011

17 Pediatric Classifications (Panama 2011): General Categories 1. Prenatal or developmental pulmonary hypertensive vascular disease 2. Perinatal pulmonary vascular maladaption (PPHN) 3. Pediatric cardiovascular disease 4. Bronchopulmonary dysplasia 5. Isolated pediatric pulmonary hypertensive vascular disease (isolated pediatric PAH) 6. Multifactorial pulmonary hypertensive vascular disease in congenital malformation syndromes 7. Pediatric lung disease 8. Pediatric thromboembolic disease 9. Pediatric hypobaric hypoxic exposure 10. Pediatric pulmonary vascular disease associated with other systemic disorders Del Cerro et al, PVRI Pediatric Taskforce, Panama 2011

18 Rivera L, et al. Front Pediatr 2016; 4: 33.

19 O Conner MG, Cornfield DN, Austin ED. Curr Opin Pediatr 2016; 28(3):

20 Mourani, P. & Abman, S. Clinics in Perinatology 2015; 42(4):

21 BPD Case Study: AC Ex 25-week infant (hx of PROM 22 weeks gestation) Transferred at 4 months of age (42 weeks corrected) for severe PH At time of transfer: echo with systemic-to-suprasystemic RVp at OSH PH treated with ino, inhaled epoprostenol, and sildenafil no hx of cardiac cath 5L HFNC 30% - cbg 7.36 / PCO2 68 / PO2 39 / HCO3 38 PO/NG fed

22 BPD Case Study: AC Concerns? First steps? Increase respiratory support -> CPAP -> BiPAP Wean epoprostenol Repeat echo -> improvement (>50% systemic RVp based on septal position) Wean ino Repeat echo -> continued improvement PH work up Echocardiogram, CXR, EKG Chest CTA

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25 BPD Case Study: AC Echocardiogram normalized with adequate ventilation Sildenafil was only PH therapy that was continued NPO due to risk of aspiration Tracheostomy and GT placement tolerated well Avoided cardiac catheterization Doing well at home on mechanical ventilation

26 AHA/ATS Guidelines: BPD Screening by echo is recommended Evaluate and treat lung disease prior to initiation of long-term PH therapy Aspiration, structural airway disease, adequate resp support Cardiac cath prior to long-term PH therapy Assess disease severity, LV diastolic dysfunction, shunts, pulmonary vein stenosis, systemic collaterals Supplemental O2 to maintain sat 92-95% ino can be effective with established BPD and symptomatic PH Serial echos recommended to monitor response to PH therapy

27 Pediatric Pulmonary Hypertension Network (PPHNet) Consensus Recommendations for BPD-PH The Journal of Pediatrics , e1DOI: ( /j.jpeds ) Figure

28 Questions for Discussion: Do you have PH screening guidelines for patients with BPD? How often to do you screen? Do you have a dedicated team following infants at risk of PH? Do you perform cardiac catheterization prior to starting therapy?

29 References: Abman, SH, Hansmann, G, Archer, SL, et al. Pediatric Pulmonary Hypertension Guidelines from the American Heart Association and American Thoracic Society. Circulation. 2015;132: Frank, BS & Ivy, DD. Diagnosis, Evaluation and Treatment of Pulmonary Arterial Hypertension in Children. Children. 2018;5(4). Krishnan, U, Feinstein, JA, Adatia, I, et al. Evaluation and Management of Pulmonary Hypertension in Children with Bronchopulmonary Dysplasia. Journal of Pediatrics. 2017;188:24-34.