Morning Report

Lindsey Gakenheimer-Smith, MD PGY3

 Case

 HPI: 3 yr old F presenting with 3 weeks dry cough and dyspnea

 Pertinent positives – chest pain, dyspnea on exertion, fevers, good
appetite
 Pertinent negatives – emesis, diarrhea, dysuria

 PMHx: congenital mitral valve dysplasia

 Medications: some medications for her heart (Mom can’t

remember their names)

 Allergies: none

 Surgical Hx: none

 Birth Hx: term, SVD, no complications

 Case

 Growth Hx: poor weight gain

 Development Hx: unable to unbutton clothes, kick or

throw a ball, normal speech

 Family Hx: 2nd of 3 children, no family history cardiac or

pulmonary diseases

 Social Hx: lives at home with parents, siblings, parents are

farmers

 Review of Systems: nothing pertinent

Case
 VS: T 38.5C, HR 154, RR 52, SpO2 77% RA

 Weight 12.4 kg (32%ile), Height 87 cm (32%ile)

 Pertinent Exam Findings:

 General: ill-appearing, small for age, mild respiratory distress
HEENT: MMM, no conjunctival pallor or conjunctivitis, no
oropharyngeal erythema, no LAD
CVS: tachycardic, III/VI SEM at 4th L ICS mid-clavicular line
radiating to axilla, prominent S2
 RESP: tachypneic, subcostal retractions, diffuse crackles
bilaterally
ABD: distended, liver edge 6cm below RCM, Stage 1
splenomegaly
MSK: muscle wasting, mild bilateral LE edema
 Labs/Imaging
 CBC – notable for WBC 32

 BUN and Cr – 24 and 0.4

 Electrolytes – Na 132, K 5.0, Cl 91

 UA – normal

 BCx x3 sent

 CXR – cardiomegaly, pulmonary edema, right lower lobe

pneumonia
Diagnoses

 Diagnoses
 Pneumonia
 CHF
 Mitral regurgitation
 Pulmonary HTN
 Malnutrition
 Definition (Adults)

 Pulmonary Hypertension (PH) – mean pulmonary arterial

pressure ≥25mmHg at rest on right heart catheterization
(RHC)

 On echocardiogram, estimated by
 Tricuspid regurgitation velocity (TRV) >3.4 m/sec
 RV systolic pressure (RVSP) >35 mmHg, in the absence of
right heart failure or pulmonary stenosis
 Further evidence on echocardiogram – interventricular
septal flattening, RV hypertrophy
Definition - Pediatrics

 Pulmonary Hypertension (PH) – mean pulmonary arterial

pressure (mPAP) ≥25mmHg on RHC after 2-3 months of
age

 However, mPAP often not an accurate reflection of PH in

children with CHD

 Definitive diagnosis determined by estimated mPAP,

other measurements obtained in the cath lab or on
echocardiogram, and clinical picture
 Common Causes of PH
 Infectious Diseases
 TB, chronic viral hepatitis, schistosomiasis, rheumatic heart
disease

 Hereditary Hemoglobinopathies (eg. Sickle Cell Disease,

Thalassemia)

 Untreated CHD (end result – Eisenmenger Syndrome)

 Left heart failure

 BPD

 PPHN

 Genetic Syndromes – Trisomy 21, DiGeorge, Noonan,

Scitimar
 Pulmonary Hypertension
Left CHF INCREASED L R shunts
mitral valve disease PRESSURE

Thromboemboli Diffuse damage

Alveolar hypoxia ARDS
Emphysema Preemies
Altitude Pulmonary fibrosis
Ventilatory Hamman-Rich
problems Syndrome
Fibrosis Scleroderma
ARDS Lots of others
Sleep apnea Fenphen
Lots more Primary idiopathic

Pathogenesis
Pulmonary Hypertension
Clinical Presentation

 Symptoms – dyspnea (especially with exertion), cough,

fatigue, palpitations, exertional chest pain

 Signs – increased intensity of P2, S2 narrowly split or not

split in respiration, S4, L parasternal heave

 Evidence of right heart failure - elevated JVP, peripheral

edema, wide split S2, S3, hepatomegaly, ascites, pleural
effusion, murmurs of tricuspid regurgitation and/or
pulmonic regurgitation
 Labs/Imaging Studies
 Labs – depends on presumed cause of PH
 CBC, ABG/CBG, Electrolytes, BNP, HIV, autoimmune serologies,
AST/ALT, Bili, etc.

 Imaging
 Chest X-Ray, ECG, Echocardiogram

 Cardiopulmonary Exercise Test

 Definitive diagnosis made with right heart catheterization

(RHC)

 If etiology of PH unknown, may need tests of sleep study,

ventilation-perfusion scan, pulmonary function tests, etc.
Chest X-Ray
 Treatment
 Treat Underlying Cause (doesn’t always reverse PH)

 Oxygen, inhaled NO

 PDE5 inhibitors (ie. sildenafil)

 If CHF with fluid retention present, can use diuretics (don’t

overdiurese, monitor electrolytes)

 If pulmonary vessels reactive in cath lab, can use calcium

channel blockers

 Prostacyclin pathway agonists (epoprostenol, iloprost)

 Initial Management

 Placed on ½ LPM NC – SpO2 now 97%

 Started cefotaxime

 Continued home captopril, aldactone, furosemide

 Enrolled her in hospital nutrition program

 Consulted cardiology – although cardiologist only comes

once/month

 Echocardiogram? – if cardiologist is around

 Other medications to treat PHN – many aren’t available in Rwanda

 Definitive treatment - Surgery – in Rwanda only performed by

humanitarian teams (~20-30 total cardiac surgery cases yearly)
 Complications

 Right Heart Failure (RHF)

 Increased demand on right heart due to elevated
pulmonary pressures

 Pulmonary Hypertensive Crisis

 Acute increase in pulmonary arterial pressure and
pulmonary vascular resistance -> RHF, often with shock +/-
myocardial ischemia
 Caused by acute hypoxia, acidosis, pain, anxiety, other
stressful stimuli
 Most common after surgeries (especially cardiac) or acute
withdrawal of PH medications
 Conclusion
 Pulmonary Hypertension – mean pulmonary arterial pressure
≥25mmHg at rest on RHC

 On echocardiogram, estimated by
 Tricuspid regurgitation velocity (TRV) >3.4 m/sec
 RV systolic pressure (RVSP) >35 mmHg, in the absence of right heart
failure or pulmonary stenosis

 Signs/Symptoms – dyspena on exertion, exertional chest pain,

cough, increased intensity of P2, peripheral edema,
hepatomegaly

 Treatment – treat underlying cause +/- pulmonary vasodilators

 Monitor progression with echocardiogram, RHC

 Important to counsel patients on possible limitations with exercise

or pregnancy and the importance of infection prevention
 References

 Abman, SH, et al. ‘Pediatric Pulmonary Hypertension.’

Circulation. 2015 Nov. 137(17):1-66.

 Rubin LG, et al. ‘Clinical features and diagnosis of pulmonary

hypertension in adults.’ UptoDate. 27 Feb, 2017. Accessed
online 23 April, 2018.

 Hopkins W, et al. ‘Treatment of pulmonary hypertension in

adults.’ UptoDate. 23 March 2018. Accessed online 23 April,
2018.

 Klings ES, et al. ‘Pulmonary Hypertension associated with sickle

cell disease.’ UptoDate. 15 July 2016. Accessed online 23 April,
2018.