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25 Cards in this Set
- Front
- Back
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What are causes of pulm htn?
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RAIVE: Respiratory (ILD, BPD, CDH, hypoplasia; CFCOPD, OSA)
Arterial (PAH): sporadic, familial, CHD, portal htn; HIV -Inflammatory: sarcoid/histiocytosis, fibrosis) -Venous: (left atrial, LV or L valve heart diz) -Embolic: PE |
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What protein receptor has a role in pulm htn?
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Bone morphogenic protein rector 2 (BMP-R2): involved in modulation of smooth m. cell growth and survivial
-genetic mutations in BMPR2 ~ pulm htn; leads to destabilization of pulm vasc intima, uncontrolled proliferation of endoth cells |
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What’s pathophysio of pulm htn ?
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-impaired vascular growth
-endothelial dysfunction: oxidant stress, abnl growth factor levels or impaired respoinse to GF: -increased endothlin 1 (ET1), serotonin (5-HT) -decreased nitric oxide (NO), prostacyclin (PGI2) -mutations in BMPR2, endolglin, alk01 eNOS, ET1; |
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What kind electrolyte imbalance occurs?
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b/w K and calcium channel activity resulting in smooth muscle cell fibroblast proliferation
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What are consequences of PPHN?
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Increased PVR, R to L shunting; (at atrial or ductal) and severe hypoxemia
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What are consequences in NB of persistent pulm htn?
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Get transient responses to inhaled NO and epo;
-when results from neon lung diz as mec asp; pulm vasc changes are most severe in regions of lung showing greatest parenchymal damage |
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Kids with IPAH have what kind of cardia function?
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Normal cardiac index (vs adults, have low cardiac index)
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What’s normal physio of pressures after birth?
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normally PVR decreses after birth, reaches NL adult levels by 4-6 wks llife. Normally, pulm vasc bed is a low pressure, low resistance, highly distensible system that can accomadate large increases in pulm blood flow w/ minimal elevations in PAH.
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What are sx of pulm htn?
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Dyspnea, fatigue, inab to increase CO w/ exercise; unexplained hypoxemia)
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Whats patho phys of PUlm htn?
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Increased PVR leads to increase in PAP at rest, then RVH (due to increased afterload), then LVH; pulm vasc disease, then RV failure: worst sign
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What’s more common sx in kids?
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Syncopal episodes; often exertional or after: decreased cerebral blood flow; can be exacerbated by peripheral vasodilation during PE
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What are sx in infants?
Kids? |
: signs of low CO: tacyhpnea, taccy, poor appetite, FTT, lethargy, irritability, crying spells (chest pain)
- older: cyanosis w/ exertion (due to R—L shunting) thru PFO or CHD -older: syncope, exertional dyspnea, Chest pain (from RV ischemia) -peripheral edema-sign of RV failure, ~ pulm vascular diz |
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What are PE of pulm htn?
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-RV failure; loud second heart sound (pulm component
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What’s CXR show w/ pulm htn?
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Mayb clear lung fields, w/ decreased pulm vascular markings; dilated cardiac silhouette;
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What tests should be done to eval pulm htn?
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Echo: exclude CHD:
Also cath: to measure the severity, evaluate for pulmonary venous obstruction or left heart dysfx and help guide decision on appropriate therapy |
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If abnl of oxygenation, abnl cxr, or PFT, what test shud be done?
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High resolution CT to rule out interstitial lung diz; if abnl; get lung bx
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What’s criteria for defining pulm htn?
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Incrase in pulm art pressure and secondary RV failure; Mean pulm art pressure > 25 at rest or > 30 with exercise
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How dx pulm htn/see response via cath?
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signifcant response to acute pulm vasodilator testing (Iv epoprostenol, iNO, adenosine, or inhaled iloprost) is considered a reduction in Mean pulm artery pressure of at last 20% w/ no change or an increase in CO. ( those who acutely deteriorate or deompensate may happened w/ empriric CCB in pts who not acute responsive , esp those w/ lung diz)
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What’s normal PCWP?
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8-12
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How does NO work?
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Vasodilator, made by NOS in endothelium; activates GC (guanylate cyclase) to increase cGMP results in activation of calcium efflux and vascular smooth muscle cell releaxation;
-also improves ventilation/perfusion matchting of ventilation and improves oxygenation -during heart cath, if pt responds to vasodilators, then pt shud be placed on CCB |
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What meds increase c AMP and cause vasodilation, inhib smooth m. proliferation?
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Prostacyclin analogues: flolan, iloprost, remodulin; also inib platelet aggregation, endothlin receptor antagonist
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What causes increase in calcium and vasocaonstricion, and smooth m. proliferation ,and which meds act here?
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Endothlin 1 causes this;
** meds that block this effect: ET- receptor blockers: bosentan, sitaxentan, ambrisentan -decrease pulm and systemic vasc resistance leading to increased CO w/o increasing H.R |
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What degrades cGMP, and what meds act on this?
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PDE5 (phodiesterase inhib);
-meds that block this: sildenafil, diprydamole, zaprinast |
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When is CCB used?
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Only in “responders to vasodilators testing during cath;
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What are SE of CCB?
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Hypotension, PE, RVF, death
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