3. -Reasonably well 6 months back
-SOB : Exertional, Initially NYHA 2
-Gradually progressive
-NYHA 3
-Associated with PND
-Orthopnia
-No seasonal or diurnal variation
-No history of allergy to cold, dust, fume or food
3
4. - Evaluated in multiple center
-Treated with oral & injectable medicines
-Not completely improved
- NYHA 4 for 2 weeks
-Admitted to multiple hospitals
4
5. Immediate Bed Side clinical examination and Resuscitation process
Started
5
General
Examination
Pectus Excavatum
Visible Apical Impulse
Apex beat 7th ICS
PP2, Heave +
Systolic thrill at Apex & left
sternal edge
PSM at Apex (5/6) & Left
sternal edge (5/6)
Distressed
Malnourished
Decubitus: Sitting and leaning
Pulse – 110 bpm
BP – 100/70 mmhg
RR – 24 breaths/min
Ankle Edema ++
JVP – Raised (6cm)
Anemia –
Cyanosis -
Systemic
Ex:
13. Acute on CHF (HFpEF)
MR (MVP)
Pulmonary Hypertension (Severe)
14. Cause of MVP ?
Mitral valve prolapse (MVP) refers to a systolic billowing of one or both mitral
leaflets into the left atrium during ventricular systole (valve prolapse of 2
mm or more)
Primary MVP occurs with increased frequency in patients with Marfan
syndrome and other connective tissue diseases (Ehlers-Danlos syndrome,
osteogenesis imperfecta, or pseudoxanthoma elasticum)
[Mitral valve incompetence: epidemiology and causes
Vol. 16, N° 11 - 16 May 2018, ESC e-journal of cardiology practice]
16. Dermatosparaxis type (dEDS)
Patient with dEDS (formerly EDSVIIC) tend
to show a set of common body features.
These include a
-short stature and finger length,
-loose skin of the face, with comparatively
full eyelids,
- blue-tinged scera,
-skin folds in the upper eyelids,
-downward slanting outer corners of the
eyes (palpebral fissures)
-small jaw (micrognathia).
17. Diagnosis by Genetic testing
`At least 20 genes have been found to cause the Ehlers-
Danlos syndromes. (Variants in the COL5A1 or COL5A2
gene, or rarely in the COL1A1 gene).
Management of EDS:
Not curative. Only supportive