pulmonary hypertension kruti jambula 4.15.11. definition ph when a disease elevates pulmonary...
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Pulmonary HypertensionKruti Jambula
4.15.11
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Definition
PH when a disease elevates pulmonary arterial pressure above normal.
Pulmonary arterial pressure = LA pressure+ (Pulmonary blood flow x Pulmonary Vascular Resistance)
PA pressure >25mm Hg is abnormal.
Often progressive, if untreated RV dysfunction.
Prognosis depends on reversibility of underlying disorder.
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Classification
WHO classification, initially proposed in 1998 and revised in 2003 categorizes based on pathophysiology, clinical presentation and treatment.
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Classification
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PPHN
Most common cause in newborns.
0.2% of all term infants.
With or without acute respiratory conditions -> persistently elevated pulmonary vascular resistance -> R to L shunting across Foramen ovale/DA -> significant hypoxemia.
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• In the fetus systemic and pulmonary arterial pressures are almost equal because of a highly vascular placenta for gas exchange and constricted pulmonary vessels.
• At birth PAP decreases to 50% systemic and PBF increases by almost tenfold.
• Decline in pulmonary vascular resistance greatest in first 24 hours and continues to fall in the first 2 postnatal weeks.
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Multifactorial increase in PBF
• Increased arterial PH and oxygen tension
• physical pulling open of capillaries accompanying lung inflation
• local endogenous vasodilatory mediators- PGs and NO
• removal of the low systemic vascular resistance placenta following clamping of UC.
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• Most newborns that have PPHN have maladaptation despite normal pulmonary arterial number and muscularization.
• Mediated by a complex imbalance in local vasodilatory and vasoconstrictor metabolites- NO, PG, TAX, LKT, BK and inflammatory cytokines.
• Chronic in utero hypoxia leading to increased medial muscle thickness, obstruction accompanying polycythemia or TAPV connection, pulmonary overflow following ductal narrowing, decrease in pulmonary arteries following pulmonary hypoplasia and CDH.
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Presentation of PPHN
• Profound and labile hypoxemia, out of proportion to parenchymal disease- suggestive, not diagnostic.
• acutely ill in DR or gradually escalating signs - cyanosis, grunting, flaring, retractions, tachypnea, tachycardia and shock.
• precordium normoactive unlike CHD.
• S2 single and loud, TR systolic murmur.
• BP/ perfusion- normal or cardiogenic shock.
• Hypoxemia and acidosis constricts pulmonary vascular SM further- vicious cycle.
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• If shunting exclusively at Ductus: pre and post ductal Pao2 gradient of greater than 20mm Hg and a similar gradient in oxygen saturation with a decrease in postductal of greater than 5%.
• Absent gradient because of shunting at atrial level or intermittent shunting.
• XR: underlying lung disease or remarkably clear with diminished vascular markings and a slightly dilated heart- Idiopathic PPHN.
• EKG- Normal
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• Echo: exclude cyanotic heart disease.
• R to L shunting across foramen ovale or DA.
• Deviation of atrial septum from R to L.
• RA enlargement and TR.
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Treatment
• Aimed at preventing end-organ injury from hypoxia, ischemia and barotrauma.
• Correct any contributing disturbances: hypoglycemia, polycythemia, hypothermia or pneumothorax.
• Maintain systemic vascular resistance: colloids/crystalloids and inotropes
• Lower pulmonary vascular resistance: iNO which activates soluble guanylate cyclase and increases cGMP-> activation of cascade causing calcium efflux and vascular SM relaxation.
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• iNO:
• activates soluble guanylate cyclase and increases cGMP-> activation of cascade causing calcium efflux and vascular SM relaxation.
• Rapid deactivation by reduced Hb leads to decreased systemic effects.
• Improves oxygenation and reduces need for ECMO by ~40%, no decrease in mortality.
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• ECMO: Only the sickest patients are now referred.
• Delay in starting ECMO, longer durations, increased age at initiation, increased rates of ECMO complications.
• Survival varies with cause of PPHN.
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Outcomes of PPHN
• Depends on etiology, severity of hypoxemia and resultant encephalopathy.
• Maldevelopment of pulmonary parenchyma and vasculature have worse prognosis.
• Survivors have an increased incidence of neurodev. impairment, neurosensory hearing loss, behavioral problems and respiratory difficulties.
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PH in Infants and Children
• Most common causes are CHD and Pulmonary disease.
• signs and symptoms are nonspecific, maybe overshadowed by underlying disease process.
• Dyspnea on exertion and fatigue that progress because Right heart cannot increase CO.
• Signs of right heart failure and Syncope on exertion.
• Death from hypoperfusion of subendocardial tissue due to increased wall stress and increased myocardial demand or from compression of L MCA by an enlarged pulmonary artery.
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Diagnostic studies
• EKG: Evidence of RVH or Cor pulmonale.
• 2D Echo with Doppler: Confirmatory test.
• Tricuspid valve regurgitant velocity + RA estimated pressure = RV Pressure.
• In the absence of PV stenosis this equals PA pressure.
• Indicators of severity: degree of RV pressure elevation and reversibility.
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Cor Pulmonale
• Alteration in right ventricular structure and function due to PH caused by disease affecting the lung or its vascular bed.
• Does not include left sided heart failure.
• Rule out CHD or acquired left-sided heart disease prior to diagnosis.
• If RVH seen on CXR or EKG - 2D Echo to diagnose.
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Congenital Heart disease
• Pulmonary blood flow is increased, or some other factor increases downstream resistance to blood flow through lungs. (MS, PV obstruction, LV dysfunction)
• PAH most common mechanism.
• Significant shunt from systemic to pulmonary circuit.
• Intracardiac: ASD/VSD/endocardial cushion defects
• Extracardiac: PDA, Aortopulmonary window
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Adapted from Nadas AS, Fyler DF. Pediatric cardiology. Philadelphia: WB Saunders, 1972:684,
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Structural features quantified in CHD
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• L to R shunt -> Increases flow in pulmonary vascular bed -> Pulm. HTN
• Shear stress on endothelial wall -> Pulm. Arteriolar endothelial dysfunction -> Arteriolar beds’ SM proliferates and hypertrophies -> PH irreversible and progressive.
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Heath–Edwards classification of pulmonary vascular changes.
Grade I: Medial hypertrophy.
Grade II: Cellular intimal proliferation in an abnormally muscular artery.
Grade III: Occlusive changes. Medium is thickened as a result of fasciculi of longitudinal muscle, and vessel is all but occluded by fibroelastic tissue.
Grade IV: Dilation. Vessel is dilated, and medium is abnormally thin (arrow). Lumen is occluded by fibrous tissue.
Grade V: Plexiform lesion. There is cellular intimal proliferation (arrow); clustered around are numerous thin-walled vessels that terminate as capillaries in the alveolar wall
Grade VI: Acute necrotizing arteritis.
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Eisenmenger Syndrome
• Once pulmonary pressure exceeds the systemic pressure-> L to R shunt reverses -> Cyanosis
• Supportive care only therapy.
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Pulmonary Venous Hypertension
• Left atrial or ventricular disease, left sided valvular disease and pulmonary venous obstruction.
• LV disease -> increase LVEDP ->primary LV failure.
• Uncommon.
• Viral myocarditis
• Medical treatment to transplant.
• PV stenosis is irreversible.
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Idiopathic PH
• Primary Progressive elevation of PA pressure ->RV failure.
• Rare with a female preponderance 1.7 : 1
• 6- 10% are familial, AD inheritance, BMPR-2 mutation
• Pathogenesis:
• Vasoconstriction due to an imbalance of mediators
• Vascular remodeling due to proliferation of endothelial cells and vascular SM.
• Thrombosis due to coagulation abnormalities.
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• Treat pulmonary vasculopathy and synproms of RV failure and thrombosis.
• Catheterization of right heart if targeted vasodilator therapy.
• Atrial septostomy-> obstructed pulmonary vascular bed bypassed-> increased CO.
• i NO responders-> CCB
• i NO non responders ->PG analogs
• Bosentan: inhibition of potent endogenous vasoconstrictor endothelin.
• Sildenafil : Breakdown of vasodilator c GMP
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Respiratory disorders
• Hypoxemia ->remodeling of vascular wall->increased resistance->RVH-> RHF
• Alveolar hypoventilation syndromes assoc. with thoracic cage abnormalities.
• OSAS, Polycythemia
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Alveolar capillary dysplasia
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Thromboembolic disorders
• Obstruction of Pulm. Arteries by a venous clot.
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References
• Rothstein et al. Pulmonary Hypertension; Peds Rev. Vol 30, No.2 38-47.
• Moss and Adams, Textbook of cardiology.
• Pulmonary Arterial Hypertension: Harrison W. Farber and Joseph Loscalzo,NEJM 2004; 351:1655-1665