26 May 2022

9/2/20: Pulmonary Arterial Hypertension

PGY2 Stefano Iantorno presented a case of a 17 y/o male with history of HTN, obesity and learning disability presents to the ED for 2 episodes of syncope.  The first syncopal episode occurred 3 weeks prior to admission.  Per family’s report, patient was running in PE class when he suddenly felt dizziness, chest pain and fell to the ground.  Fall was witnessed by other students who denied that the patient had any shaking or posturing, urinary incontinence or tongue biting.  The patient did have loss of consciousness for unknown duration of time, unclear if he hit his head or not.  The second syncopal episode occurred 5 days prior to admission.  The patient was dancing at a family part and suddenly felt dizziness and chest pain again, was helped into a chair and briefly lost consciousness for 3 mins.  Denies any abdominal complaints, decrease in appetite, PO intake or recent illnesses/exposures.  Home medications include amlodipine.  Family history only significant for maternal grandmother with DM and MI.  He was born in El Salvador and moved to LA 5 years ago.

Vital signs with T 37, HR 77, BP 116/65, RR 22, satting 98% on RA, BMI 34.  Exam notable for normal S1, fixed split S2, no murmurs, lung clear to auscultation bilaterally, no edema in b/l LE.

CBC: WBC 7.6 > Hb 18.2/Hct 51.6 <Plt 242


Na 141/K 4.2/Cl 104/HCO3 20/BUN 12/Cr 0.8 <Glucose 99

ALP 145>T.protein 7.7/Albumin 5/AST 26/ALT 35/Tbili 0.3

Coags:PT 13.1, INR 1.01, PTT 29.5

Orthostatic vital signs was negative for orthostasis.



Cards/Pulm workup:

  • ABG: 7.43/82/27/18 on 21% FiO2
  • D-Dimer 0.35 (normal <0.49)
  • Troponin <0.01
  • BNP 291 (normal <125)
  • Utox negative
  • Inpatient sleep study done and patient passed without needing additional support at night
  • TTE/TEE showed dilated RA, dilated MPA, flattending with R to L bowing (D shaped LV) of the ventricular septum with paradoxical septal movement.
  • CT Cardiac angiogram significant for flattening of the interventricular septum, R ventricle dilation with hypertrophy and R atrium is dilated.  There is dilated main pulmonary artery measuring 3.6cm.  Negative for ASD.
  • V/Q scan was negative
  • CT Hi Res Chest showed enlarged main pulmonary artery consistent with pulmonary arterial hypertension and subtle centrilobular and perivascular ground glass attenuation is nonspecific.

Rheum workup:

  • ANA negative
  • ESR 2 mm/hr
  • CRP 0.8 mg/L
  • CK 101 U/L

ID workup:

  • UA negative for protein, leuks, nitrite or blood
  • HIV negative
  • Schistosomiasis Ab negative

Right heart cath was done which showed the following pressures:

  • RA 11
  • RV 88/5
  • PA 85/35 (53)
  • PCWP 20
  • Transpulmonary gradient 40-45 mmHg
  • No change in hemodynamics or outputs after inhaled nitric oxide challenge.

Treatment: Patient was transferred to the MICU and started on Milrinone gtt and Remodulin gradually uptitrated to goal of 30 ng/kg/min with no side effects.  Milrinone was discontinued due to stable cardiac input and sildenafil 20mg Q8H was started.  He was subsequently transferred to OSH for further maintenance therapy.

Take home points:

Diagnostic criteria: RHC with PAP >25mmHg NO – test vasoreactivity  
Group 1Pulmonary arterial hypertension
Hereditary (worse outcome)
Drug and toxin
Liver disease (portopulmonary htn)
Congenital heart disease
Pulmonary capillary hemangiomatosis (need lung biopsy)
HIV Schistosomiasis
CCB – PO mediation; use if reactive to NO in RHC

Prostanoids (Epoprostenol, Treprostinil – Remodulin)

Endothelin-1 receptor antagonists (Bosentan, Ambristentin) – PO medication; liver toxicity, teratogenic  

PDE-5 inhibitors (Sildenadil, Fadalafil) – PO medication
Group 2Cardiac
Heart failure
Valvular failure
Group 3Lung
Restrictive disease: ILD
Developmental lung disease
Hypoxia w/o lung disease
Sleep study
PFTs (decreased DLCO without other abnormalities)
Group 4Chronic thromboembolismCTPA, V/Q scan Abnormal coag workup
Group 5Heme disorders (MPD, SCD) Systemic and metabolic disorders (Sarcoid, chronic renal failure, glycogen storage disease, neurofibromatosis, etc) 

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