Dr. Devendra Patil

IMCU Chief:- Dr. Dhandapani Unit

CASE PRESENTATION

Munuswamy, 24/M was brought to the IMCU with complains of :-Progressive swelling of all 4 limbs 3 daysIncreasing difficulty in breathing 3 daysSevere Respiratory Distress 6 -7 hrsHOPI:-

Not asso with facial puffiness, Not aso with jaundiceNot asso with feverNot asso with cough with expectorationNot asso with haemoptysisNot asso with abd distensionNo h/o similar episodes in pastNo h/o prolonged immobilisationNo h/o major surgeriesNo h/o swelling of veins of the legsNo h/o crushing pain ,redness , swelling of lower limb calf

Personal History:Manual LabourerNot a K/c/o SHT / DM / PTB / CAD / RHDSmoker and Alcoholic

On Examination:Pt was conscious oriented AfebrileTachypneic with distressPR – 140/min feebleBP – 80/40 mm HgRR – 29/minSpO2 – 60 % without O2

improved to 95 % with O2 facemaskRS : -Trachea centralRespiratory Distress +ntUse of Accessory Muscles +ntIntercostal Retractions +ntNo e/o spino – scapular or shoulder abnormalitiesNo e/o asymmetry in chest wall movements

No Pallor icterus clubbing cynosis +nt oedema involving all 4 limbs +nt No e/o facial puffinessdistended Neck Veins

RS ( Contd…)Dull note +nt in R. infra axillary infrascapular areasTidal percussion +nt in 4th ICSOther areas were resonant

Auscultation: Reduced air-entry in R. infraaxillary infra scapularBronchial Breath sounds in L. Interscapular regionFew crepitations present in R. infra axillary areas

CVS:Apex Beat in 5th ICS medial to MCL hyperdynamicNo e/o of any visible PulsationsJVP – elevatedSystolic thrill palpable in Pulmonary areaPalpable P2Mitral Area : s1 s2 heard. No added sounds. No murmurPulmonary Area: ejection systolic murmur.Carvallo’s sign +ve

CNS :- No E/o FNDP/A :- Soft non tender no organomegaly

‘

Investigations :-

Hb 10.4 gm%

TC 7800 cells/ cc

DC P64/ L32/ E2

Platelet 2.3 lakh

ESR 12 mm /hr

PCV 37%

MCV 86 %

MCHC 32%

MCH 34%

RBS 143 mg%BUN 32 mg %S. Cr 0.6 mg %Na + 133 meqK+ 3.2 meq

S. Bili (T) 1.1 mg %S. Bili (iD) 0.6 mg%SGOT 39 IUSGPT 27 IUALK PHOS 42 IUAPTT 24 secPT 14 sec

X-Ray Chest:R-sided Mild Pleural EffusionCardiomegaly

I

II

III

aVR

avL

ECG FINDINGS:-

1. Sinus rhythm2. R-axis deviation3. Low voltage complexes4. Broad P waves5. R/S ratio high in v16. T inversion in V1 V27. ST depression in V2 V38. No specific S1Q3T3 pattern

Ecg impression:-

R- atrial enlargementR-ventricular enlargementPericardial effusion

IMPRESSION :

C/o Acute onset Right Ventricular Failurewith Pulmonary Hypertension.

Echo findings :

RA DilatedRV DilatedRV free wall Hypokinesia +nt ( Mc’Conell sign +nt )Severe PHTTR mildTRPG 75 mm LVEF – normalMain Pulmonary Trunk – DilatedInter Atrial Septum bulging into LAMild to moderate Pericardial Effusion

Doppler study of Leg Veins :

Normal. No e/o DVT.

PROVISIONAL DIAGNOSIS:-

ACUTE PULMONARY THROMBO-EMBOLISM of ? origin

Course in IMCU :-Pt was started on Inj. Heparin 5000 IU iv bolus followed by

1000 IU iv/hr as continous infusionPt continued to remain Hypotensive and Dysnoeic even on

2nd day of Ionotropic support and Oxygen.

ECHO was reconsidered2nd ECHO findings:-RA and RV dilatedMod TRPG 70Severe PHTNo e/o clot in MPAMPA RPA LPA moderately dilatedModerate pericardial effusion

Diagnosis :

1.Pulmonary Thrombo-embolism2.Primary Pulmonary Hypertension

Suggested Investigations:-

HRCTCT – Angio.D- Dimer AssayHyper- Coagulable states PROFILEHIV Elisa RA , ANA , APLA , ACLA , ANCA titres ( CTD Screening )USG ( abdomen ) ABG analysisPFT s

Further Course of Illness in IMCU:-HRCT couldn't be done as pt was too dysneic and hypotensive.Pt had a severe cardio – respiratory arrest and succumbed to his illness on the 2nd day night.

PULMONARY HYPERTENSIONPulmonary hypertension-any condition the PA pressure at rest is

consistently >35 mmHg systolic, 15mm Hg diastolic or 25mmHg mean, or exertional mean PAP >35 mm Hg with normal resting values.

Severity of Pulmonary HypertensionDegree of disease

Mild

Moderate

Severe

Mean PAP (mmHg)

25 - 40

41 - 55

>55

MPA = CO * PVR + PCWP

Congenital Heart Defects Congenital ShuntsEisenmengerization(rare) Beri beri(rare) myeloma

Usually due to left-sided heart disease (valvular, coronary or myocardial), obstruction to blood flow downstream from the pulmonary veins

WHO World Symposium, Venice 2003 PAH Classification

I. Pulmonary arterial hypertensionFamilialIdiopathic (formerly called primary)Related to:

Collagen-vascular diseaseCongenital heart disease, shuntsPortal hypertensionHIV infectionDrugs / toxins/otherHemoglobinopathies (Sickle cell, thalassemia)

II. PH related to pulmonary venous hypertension (left heart disease)

III. PH related to disorders of respiratory system

IV. PH caused by thromboemboliNon-thrombotic pulmonary embolism: tumor, parasites

V. Miscellaneous: Sarcoid, extrinsic compression

Treatment: Right Heart Failure

Reduce RV wall stress (MVO2, ischemia)Reduce RV afterload

○ Pulmonary vasodilators (O2, high dose CCB, ERA’s,

prostanoids, nitric oxide)○ Anticoagulation to prevent thrombosis

Reduce RV preload

(diuretics: loop, aldosterone antagonists) Improve RV inotropy

Chronically: digoxinAcutely: low dose IV dobutamine or

dopamine at 1-2 mcg/kg/min

IV PG analogues (epoprostenol treprostinil)

Approved Agents and side-effects

Class of Drug Drug Dis-advantages

ET -1 AntagonistOral Bosentan/ 62.5mg bd

Hepatic toxicity (11%;transient, reversible) C/I with glyburide , cyclosporine

PDE-5 InhibitorSildenafil Citrate (20, 40 or 80 mg tid)

Headache, flushing, dyspepsia. Avoided with nitrates

Prostacyclinanalogue

InhalationalIloprost/

Frequent administration 6 to 9 times daily. Short t1/2. flushing cough

Prostacyclinanalogue

Sc Treprostinil/ Pain, erythemaat infusion site

Prostacyclinanalogue

IV Epoprostenol/20-40 ng/kg/min

Indwelling central line and Pump(infection ,malfunction, flushing diahorrea, jaw pain

Thank – You.