Nelsons Chapter 460.1 Pulmonary Hypertension Flashcards
1
Q
PH is characterized by?
A
- pulmonary vascular obstructive disease and
- right sided HF
2
Q
PH occur at any age but mean age is?
A
7 to 10yo
3
Q
In idiopathic or familial disease PH more common in?
In other disease?
A
- Female
- Equally prominent
4
Q
Etiology familial PPH?
Idiopathic PH?
Other triggers?
A
- 70% mutation BMPR2 on chromosome 2q33
- 10-20% idiopathic sporadic PH
- viral infection like HPV 8
5
Q
Pathophysio of PH
A
- hyperplasia of muscular and elastic tissue and thickened intima of small pulmonary artery and arterioles cause precapillary obstruction of pulmonary vascular bed
- secondary atherosclerotic changes in large pulmonary arteries
6
Q
Dx of PPH
A
B4 dx can be made, other causes of elevated pulmonary pressure must be eliminated
7
Q
Other causes of elevated pulmonary artery pressure aside from PPH?
A
- chronic pulmonary parenchyma disease
- persistent obstruction of upper airway
- congenital cardiac malformation
- Recurrent pulmonary emboli
- alveolar capillary dysplasia
- liver disease
- autoimmune disease
- moyamoya disease
8
Q
Updated classification of PH?
A
- PAH
- idiopathic
- heritable
- drug and toxin induced
- assoc with connective tissue dse, HIV infxn, portal htn, CHD,
Schistosomiasis
1’. Pulmonary venoocclusive dse and/or pulmonary capillary hemagiomatosis
1’’. PPHN
- PH due to left heart disease
- LV systolic dysfunction
- LV diastolic dysfunction
- valvular disease
- congenital/acquired Left heart inflow.outflow tract obsruction and
congenital cardiomyopathies
- PH due to lung disease and/or hypoxia
- COPD
- interstitial lung disease
- other pulmonary dse with mixed restrictive and obstructive pattern
- sleep-disorder breathing
- alveolar hyperventilation disorder
- Chronic exposure to high altitude
- developmental lung dse
- Chronic thromboembolic PH (CTEPH)
- PH with unclear multifactorial mechanisms
- hematologist disorders: chronic hemolytic anemia, myeloproliferative disorders, splenectomy
- systemic disorder: sarcoidosis, pulmonary histiocytosis, lymphangioleiomyomatosis
- metabolic disorder: glycogen storage dse, gaucher dse, thyroid d/o
- others: tumoral obstruction, fibrosing medistinitis, chronic renal failure, segmental PH
9
Q
Developmental lung disease associated with PH
A
- congenital diaphragmatic hernia
- BPD
- alveolar capillary dysplasia (ACD)
- ACD with misalignment of veins
- lung hypoplasia (primary or secondary)
- surfactant protein abnormalities
Surfactant B (SPB) deficiency
SPC deficiency
ATP-binding cassette A3 mutation
Thyroid transcription factor 1/Nkx2.1 homeobox mutation - Pulmonary interstitial gylcogenosis
- Pulmonary alveolar proteinosis
- Pulmonary lymphangietasia
10
Q
PH assoc. with CHD %?
with chronic respiratory d/o?
Idiopathic or familial?
Chronic lung dse (BPD)?
A
CHD 40-55% most common
Chronic respi 20-35%
Idiopathic or familial 10-15%
BPD largest portion in new cases
11
Q
Vascular abnormalities associated with pulmonary arterial hypertension?
A
- abnormal muscularization of distal and medial precapillary arteries
- loss of precapillary arteries
- thickening of large pulmonary arterioles
- new vascular intimal formation that is occlusive in vessels <500-100uM and in plexiform lesions.
12
Q
- PH places after load burden on the _____, which results in ______.
- Dilation of the ______ is present and ____ insufficiency may occur.
- in later stage of dse, ______ dilates, _______ insufficiency develops, and _____ is decreased.
A
- PH places afterload burden on the RV, which results in RVH.
- Dilation of the pulmonary artery is present and pulmonary valve insufficiency may occur.
- in later stage of dse, RV dilates, tricuspid insufficiency develops, and CO is decreased.
13
Q
Complication of PH?
A
- arrhythmia
- syncope
- sudden death
14
Q
PH CM?
A
Predominant - exercise intolerance (dyspnea) - Fatigability Occasional - precordial chest pain, - dizziness - headaches - syncope (30%) - peripheral cyanosis (patent foramen ovale) Late stage dse - cold extremities - gray appearance associated with low CO If with right sided HF - jugular venous pressure elevated, (jugular venous a wave) - hepatomegaly - edema With functional tricuspid insufficiency - jugular cv wave - systolic hepatic pulsation - heart moderately enlarged - RV heave Dilated pulmonary artery - S1 followed by click - s2 narrowly split, loud, booming in quality, palpable at LUSB - presystolic gallop rhythm at LLSB Pulmonary insufficiency - systolic murmur is soft and short, - blowing decrescendo diastolic murmur Tricuspid insufficiency - holosystolic murmur at LLSB
15
Q
Diagnosis of PH?
A
- CXR
- prominent PA, RV
- prominent vascularity in hilar area
- decreased pulmonary markings in the peripheral lung fields. - ECG
- RVH -> spiked p waves - 2D Echo
- screen any congenital cardiac malformation
- if with tricuspid valve insufficiency
- estimate RV (PA) systolic pressure - Cardiac catheterization
- can evaluate the presence of left sided obstructive lesions that result in pulmonary venous hypertension.
