3/10/2017 SESSION 3: Pulmonary Venous disease Part I • NO DISCLOSURES Dra. Maria Jesús del Cerro Pediatric Pulmonary Hypertension Unit “RAMON Y CAJAL University Hospital. Madrid, Spain � Q1: Anatomy, Histology of the lesions. � Q2: AND CONSEQUENCES of PV Stenosis in preterm Case report: Ex preterm , Relationship betwen 22 weeks GA Relationhip between Surgery PDA PV stenosis and outcomes of Pretem babies… Dg Left pulmonary vein stenosis at autopsy performed at 23 months age � Q4: WHAT CAN WE LEARN FROM other human PV stenosis entities and from ANIMAL MODELS of PV stenosis? � SUMMARY Benjamin JT 1
3/10/2017 Case report: � Female, venous congestion Birth weight 1.600 gr Subpleural and interstitial � TOF, Pda Small PV right Lung Spindle shaped Intimal Cells � NEC needing surgery in the ridge At 5 months, diagnosed with PV stenosis of 3 veins.. Occluded lumen Autopsy at the stenosis Small PV left Lung Obliterative fibromuscular thickening BOTH IN � LARGE VEINS at the venoatrial junction Lumen of the normal, � SMALL VEINS Remodelling of proximal vein SEVERE INTERSTITIAL small peripheral artery FIBROSIS Medium hypertrophy IN THE LUNG Alveolar simplification Mc Connell, Pediatric Cardiology 1994 • Q1: anatomy, histology of the lesions 60% Unilateral 86% Left Side 40% Bilateral DISCRETE STENOSIS Pattern Dressner, Pediatrics 2008 AT THE VENOATRIAL JUNCTION at presentation 64% Unilateral 95% Left Side 36% Bilateral DIFFUSE STENOSIS OF THE “UPSTREAM” Maghoub, Ped Pulmonol 2017 PULMONARY VEINS INTO THE LUNG PARECHIMA ATRESIA of the VEIN ACQUIRED, PROGRESSIVE LESIONS 2
3/10/2017 Multicentric study : � Q2: AND CONSEQUENCES Of PV Stenosis in preterm infants? 39 expreterm Relationship betwen with PV stenosis Relationhip between PV stenosis and outcomes of Pretem babies… Maghoub L, et al PV STENOSIS AND BPD… 2008 Pulmonary Vein Stenosis: Prematurity and Associated Conditions 58 cases 26 pt EVP: 61% prematuridad 88% associated cardiac lesions. � Median GA 28 weeks EG 38% preterm babies � Median Birth Weight 750 gr. � 63% BPD 79% Cardiac Lesions � 40% documented normal veins at birth 42% DBP 28% Extracardiac syndromes � PV stenosis DG 7 mo after birth From discrete stenosis PROGRESSION to diffuse narrowing and atresia From Unilateral to Bilateral disease Development of collateral vessels to unaffected lobes /systemic veins 3
3/10/2017 20 preterm infants diagnosed with PV stenosis Associated Cardiac Lesions: 80% NEC: 50% Common embriological origin of Common embriological origin of Splanchnic Vasculature and Pulmonary Veins Splanchnic Vasculature and Pulmonary Veins VEGF mediated nflammation in NEC affecting P Veins VEGF mediated nflammation in NEC affecting P Veins NEC preceded the diagnosis of PV Stenosis… NEC preceded the diagnosis of PV Stenosis… Columbia University Maghoub, Ped Pulmonoo 2017 n % preterm % BPD Cardiac NEC 3 y lesions SURVIVAL Dressner 26 62% 48% 88% 43% Pediatrics 2008 Seale 41/58 38% 63% of 79% 49% Heart 2009 pret Heching 20 100% - 80% 50% 60% Arch Dis Child 2014 Laux 16 100% 87,5% 87,5% 12% 66% Ped Cardiol 2016 Maghoub L 39 100% 74% 100% 23% 55% Ped Pulmonol 2017 19 months age 2 months age 19 months age 4
3/10/2017 Disrupted Multicentric study : 39 expreterm with PV stenosis Lung angiogenesis 15/39 (39%) born of Edema Genetic Environmental Shunts Multiple pregnancies, factors? factors? flow in PVs With unaffected siblings Decreased Lung Inflammation Compliance EPIGENETIC citokines FACTORS …??? Lung infection, Lung infection, Sepsis Sepsis NEC NEC Chorioamnionitis Chorioamnionitis Maghoub L • HYPOPERFUSION OF THE AFFECTED LOBE/LUNG HYPOPERFUSION OF THE AFFECTED LUNG/LOBE OVERFLOW TO UNAFFECTED LOBES • OVERFLOW TO THE UNAFFECTED LUNG/LOBES • HYPOPLASIA OF THE AFFECTED LUNG • LUNG EDEMA • VENOUS COLLATERALS DEVELOPMENT • PULMONARY HYPERTENSION intrapulmonary hypoplasia of the left pulmonary artery � INFLUENCE ON THE PATIENTS OUTCOMES in left Pv stenosis/Hypoplasia 5
3/10/2017 CONSEQUENCES OF PV STENOSIS VENOUS Collaterals to systemic veins INCREASE IN PULMONARY OVERFLOW TO CAPILLARY WEDGE PRESSURE UNAFFECTED LOBES PH LEFT HEART DISEASE? ARTERIOLAR REFLEX REMODELLING ARTERIOLAR IN THE AFFECTED & VASCONSTRICTION? UNAFFECTED LOBES PAH ? LUNG EDEMA, DECREASED COMPLIANCE, INCREASE IN HYPERCAPNIA AND HYPOXIA PH DUE TO LUNG DISEASE? Courtesy of Dr Lina Caicedo and Diana M. Nuñez, Shaio Clinic, Bogotá CONSEQUENCES OF PV STENOSIS PULMONARY HYPERTENSION Multicentric study : 39 expreterm with PV stenosis 27% in a cohort of BPD with moderate/severe PH referred to a PH Unit Cerro et al, Pediatric Pulmonology, 2014 DG: CT scan /cath 4.7 % in a retrospective ECHOcardiographic Review of 213 pts with severe BPD At Ohio ´ s Children ´ s hospital Maghoub L 6
3/10/2017 n % preterm % BPD Cardiac NEC 3 y lesions SURVIVAL Dressner 26 62% 48% 88% 43% Pediatrics 2008 Seale 41/58 38% 63% of 79% 49% Heart 2009 pret Heching 20 100% - 80% 50% PV stenosis and outcomes of Pretem babies… 60% Arch Dis Child 2014 Laux 16 100% 87,5% 87,5% 12% 66% Ped Cardiol 2016 Maghoub L 39 100% 74% 100% 23% 55% Ped Pulmonol 2017 58 cases Median GA 28 weeks � Median Birth Weight 750 gr. 38% preterm babies � � 63% BPD 79% associated cardiac lesions no NEC 20 preterm infants diagnosed with PV stenosis NEC Seale et al 7
3/10/2017 AGA/LGA Multicentric study : 58 cases 39 expreterm Small for GA with PV stenosis 38% preterm babies 79% associated cardiac lesions 3 or more veins Bilateral disease Dg < 6 months age Survival 61% Survival 76% Maghoub L 16 expreterm infants from 2 French institutions Pts with Unilateral disease and 1 or 2 veins Pts with bilateral disease and 3 or more veins Laux D, et al 8
3/10/2017 SEVERE PULMONARY HYPERTENSION stent implantation in RSPV Angioplasty & stenting pulmonary veins , ANOUAR LOUBANI Balloon Angioplasty Left PVs RSPV POST stent RSPV Pre stent PROGRESSION TO Restenosis of RSPV stent Complete occlussion of left PVs Died in right heart failure PRET 27+4 WEEKS . Birth weight: 880 gr. BPD Multiple pregnacy . PDA surgically closed � Right heart failure NEC (several surgeries), Short bowel syndrome � PV stenosis at 18 months Severe BPD of Left inferior and DG with severe left PV stenosis at age 7 months Right superior PVs ECO: big ASD severe PH � Daily sincopal episodes severe gradient in left Pulmonary veins � Failure to thrive � Progressive Ph In spite of sildenafil � Pericardial effusion 5 YEARS LATER: alive, FC I-II , severe PH with triple therapy 9
3/10/2017 Age 2 y.o.: left PV stenosis (superior and inferior) Corrected TOF Limitation of agressive therapies decided…. 7 m. 7 m. Severe PH , severe GERD, HTP HTP The girl started tolerating enteral feeding.. still depending on Oxygen therapy Discharged home... Surgery for GERD Favourable evolution of the BPD… Sildenafil therapy NO intervention on the left Pvs REEVALUATED IN CARDIOLOGY AT 18 months 9 years age ASD surgically closed at 20 months age � Functional class I-II 7 years age � Mild PH (40% systemic), No residual PH � Functional class I � Sildenafil therapy � NO PH Left PV remained stenotic � Left PV remain patent � Left PV remain patent but stenotic in the CT scan but stenotic in the CT scan � Autopsies of 10 infants � Dg PV stenosis in first year life � Simple shunts Abnormal intimal Inmunohistochemical stainig Electron microscopic spindle- shaped images cellular proliferation 10
3/10/2017 3 infants developing Neointimal fibromuscular proliferation left pulmonary vein stenosis/atresia In the large central pulmonary vein In the months after open heart surgery Intimal Hyperplasia in the for complex heart disease: small intrapulmonary veins � TA+Tga+IIA type B----Norwood+Glenn � DORV +mitral stenosis– banding--Fontan Arterioles : � D- TGA+ Ao coartation--- atrial switch+Co repair Intimal proliferation Medial hypertrphy All of them had documented normal veins before surgery… 55 years old men , PH with PV stenosis, needing lower left lobectomy for recurrent hemoptisis with PV stenosis after AF radiofrecuency ablation… angioplasty, stents, surgery… Ussia GP. Eur J Cardiothorac Surg 2002;22:465-7 mPAP 4 weks old piglets PVRI RVEDV RVEDV RVEDV right Antero Sham group PV banding Lateral thoracotomy N=3 N=12 RVEF RVEF RVEF PV Banding Sham CT scan 2 months after intervention Pereda D, et al, J Cardiovasc Tras Res 2014 Pereda D, et al, J Cardiovasc Tras Res 2014 11
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