Pulmonary Hypertension

1. Pulmonary Hypertension

2. Best Method for Mx of PHTN is PREVENTION

3. Conditions associated with PAH • Acyanotic CHD • Increased Pulmonary Blood Flow • Cyanotic CHD • Increased Pulm Blood Flow

4. ACYANOTIC Increased PBF ATRIAL: ASD VENTR: VSD ARTERIAL: PDA COMBINED: VSD+PDA No Shunts Pulm or Aortic Stenosis CYANOTIC Decreased Flow TOF Pulm Atresia Increased Flow TAPVD TGA Truncus Tricuspid Atresia CLASSIFICATION OF CHD

5. So what is the right time to operate in these conditions

6. Timing of surgery: Acyanotic • ASD: 2 years or later • VSD • Large: 3-6 months • Moderate: when there is FTT • Small: when there is AI or InfectiveEndocardiaits

7. Acyanotic, when to operate • PDA • Infancy • ALL PDA’S CAN BE CLOSED WITH DEVICE • Neonatal • Prematurity • Closure by surgical ligation • Full Term • Wait for child to grow if possible

8. ATRIAL SEPTAL DEFECT PRIMUM SINUS VENOSUS

9. ATRIAL SEPTAL DEFECT-II

10. ATRIAL SEPTAL DEFECT-II

11. ASD-DEVICE

13. Acyanotic CHD • Increased Pulmonary Blood Flow • PRETRICUSPID SHUNT: RA RV DILATATION • ATRIAL SEPTAL DEFECT • POST TRICUSPID SHUNT: LA V DILATATION • VENTRICULAR SEPTAL DEFECT PATENT DUCTUS ARTERIOSUS

14. What is a Large, Moderate, Small VSD • Effects of VSD • PRESSURE EFFECT: Pulmonary Hypertension • VOLUME EFFECT: Cardiac enlargement

15. Pressure Effect: Types • Flow Related PAH: Reversible • Irreversible PAH due to permanent Changes

16. Flow Related PAH • Increased Flow • Increased Pressure • When you remove the extra flow ie close the VSD, the Pulmonary Pressure comes back to normal

17. Flow related PAH (Pre/Post Tricuspid Shunt) • Symptoms of increased Flow • Tachypnea, Rec infections, failure to thrive • Signs • Tachycardia, Harrison’s sulcus, retractions • X-ray • Cardiac enlargement, Increased Pulmonary Blood Flow

18. Increased Flow PAH • All these indicate • Patient is operable with good results without post-op PAH

19. Till when is this phase: • Reversible PAH • VSD-LARGE: UPTO 6 MONTHS • PDA-LARGE: UPTO 6 MONTHS • ASD: LARGE: UPTO LATER 4-8 YRS

20. So, What is a Large Shunt • Post tricuspid Large VSD/PDA • Clinically PAH Present (Pressure Effect) • Clinically Volume Effect Present (Cardiac Enlargement)

21. Moderate Shunt • No Pressure Effect • But Volume Effect Present

22. Small Shunt • No Pressure or Volume Effect • No Symptoms or Signs of increased flow

23. So if the surgery is done at the right time it is likely the patient will not get pulmonary hypertension

24. What Happens when • Reversible PAH starts becoming Irreversible • …the child shows some signs and these are signs of Post Op PAH

25. Signs of Reversible to Irreversible PAH • Symptoms: • Start improving • Less FTT • Less Infectios • Less tachypnea • Signs: • Murmur shorter, P2 Louder, Cardiac Enlargement less

26. When Reversible Changing to Irreversible • Patient still operable • But the post op risks are more and episode of life threatening PAH in immediate post op period is high

27. When Completely Irreversible • Patient now has Eisenmanger’s • Decreased Pulm Blood Flow • Cyanosis starts • Now risk of surgery more than living without surgery

28. Chest Xrays Indicating Increased PBF w PAH ie operability

29. VSD

30. ASD

31. MODERATE VSD

32. LARGE VSD LARGE SHUNT

33. AV CANAL

34. TGA

35. TRUNCUS

36. EISENMANGERS

37. Pulmonary Hypertension

38. Classification Group 1 PAH Examples: "Pulmonary arterial hypertension". • 1. Idiopathic (IPAH) • 2. Familial (FPAH) • 3. Associated with (APAH): • Collagen vascular disease • Congenital systemic-to-pulmonary shunts • Portal hypertension • HIV infection • Drugs and toxins • Other (thyroid disorders, glycogen storage disease, Gaucher disease, hereditary hemorrhagic telangiectasia, hemoglobinopathies, myeloproliferative disorders, splenectomy) • 4. Associated with significant venous or capillary involvement • Pulmonary veno-occlusive disease (PVOD) • Pulmonary capillary hemangiomatosis (PCH) • 5. Persistent pulmonary hypertension of the newborn

39. Classification • Group 2 PH — "Pulmonary venous hypertension". Examples: • 1. Left-sided atrial or ventricular heart disease • 2. Left-sided valvular heart disease • Group 3 PH — "Pulmonary hypertension associated with disorders of the respiratory system or hypoxemia". Examples: • 1. Chronic obstructive pulmonary disease • 2. Interstitial lung disease • 3. Sleep-disordered breathing • 4. Alveolar hypoventilation disorders • 5. Chronic exposure to high altitude • 6. Development abnormalities

40. Classification • Group 4 PH — "Pulmonary hypertension caused by chronic thrombotic or embolic disease". Examples: • 1. Thromboembolic obstruction of proximal pulmonary arteries • 2. Thromboembolic obstruction of distal pulmonary arteries • 3. Non-thrombotic pulmonary embolism (tumor, parasites, foreign material) • Group 5 PH — These patients have PH caused by inflammation, mechanical obstruction, or extrinsic compression of the pulmonary vasculature (eg, sarcoidosis, histiocytosis X, lymphangiomatosis, compression of pulmonary vessels by adenopathy, and fibrosingmediastinitis).

41. Histologically Speaking • The above mechanisms all cause small muscular arteries and arterioles to undergo intimal hyperplasia and medial hypertrophy 1 • Narrowed lumen • Decreased cross-sectional area • Increased resistance • 1 - Though again with PPH likely primary process, rather than reactive

46. PULMONARY VASODILATION • HYPEROXIA • HYPOCARBIA • ALKALOSIS • NON REM SLEEP • SEDATED • PARALYSED

47. Basic 3 Mechanisms • 2º pulmonary arterial hypertension: • Reduced cross-sectional area of pulmonary vasculature, secondary to: • Occlusion of vessels (e.g. emboli) • Primary disease of pulmonary vasculature walls (e.g. 1º pulmonary hypertension, portal hypertension) • Primary parenchymal disease (e.g. interstitial lung disease, emphysema) • Vasoconstriction 2/2 hypoxia or acidosis • Increased flow through pulmonary vascular bed secondary to left to right shunts • Increased “back pressure” secondary to pulmonary venous hypertension

48. 3 types of abnormalities Maladaptation Maldevelopment Underdevelopment

49. Maladaptation Prototype: Meconium aspiration pneumonia Pneumonia, RDS Obstruction of the airways Chemical pneumonitis Release of endothelin,thromboxane vasoconstrictors

50. Maldevelopment Prototype: Idiopathic PPHN (“black lung” PPHN) Vessel wall thickening Smooth muscle hyperplasia Cause – intrauterine exposure to NSAID constriction of ductus arteriosus genetic