Pulmonary Hypertension
Presentation
H.G., a 5-month old male presents to the PICU in respiratory distress. VS show that he is hypoxic, tachypneic, tachycardic, and febrile. BP is stable. Initial Vital Signs: HR = 200, BP = 84/35, RR = 85, Temp = 38.1 rectal, O2 sat = 84% on 2L NC
On exam, he is awake and alert, but agitated. Good pulses and perfusion. Lower extremities are slightly mottled. No evidence of cyanosis. He is noted to have severe nasal flaring and grunting with both suprasternal and intercostal retractions. Lung sounds are equal bilaterally. Rhonchi heard on auscultation. A 2/5 systolic murmur is heard.
PMH: The patient is an ex-32 week preemie with a history of BPD and GERD. He is currently on Lasix® and prednisone q-day and albuterol nebulizer prn for treatment of BPD. He is s/p Nissen and G-tube placement and taking erythromycin and Reglan® for the GERD. He is tolerating continuous G-tube feeds. Born with an APGAR of 5/10 at 1 and 5 minutes, he was intubated in the NICU and placed on mechanical ventilation. He remained on mechanical ventilation for 13 days and eventually successfully weaned to 2L NC. After 5 months in the NICU, he was transferred to the PICU for further management.
Differential Diagnosis
BPD exacerbation, sepsis, pneumonia, bronchiolitis, ALI or ARDS
Diagnosis
On arrival to the PICU, the patient appeared to be in respiratory distress. NC was increasingly titrated up with no relief in respiratory distress. He was placed on HFNC 8L 100%, which improved his oxygen saturation to 90%. He remained in respiratory distress with nasal flaring and retractions. The patient was NT suctioned and the sputum specimen sent for gram stain and culture. Labs were drawn and included, a CBC, BMP, peripheral blood culture, and an ABG. A chest x-ray was obtained. The patient was started on Zosyn® for prophylaxis. Lab results were as follows:
CBC: WBC 14.1, RBC 3.37, Hgb 10.4, Hct 29.4, Platelet 296
ABG: pH 7.38, PaCO2 64, PaO2 63, HCO3 37.9, BE 9, O2 sat 84%
BMP: Na+ 135, K+ 3.4, Cl- 98, Glucose 92, BUN 6, Cr 0.6, Ca++ 9.8, Phos 3.2, Mg++ 2
The peripheral blood culture was negative and the Zosyn® discontinued. The chest x-ray was negative. However, the patient was noted to have a slightly enlarged heart. A 12-lead EKG was ordered and WNL. A two-dimensional echocardiogram with Doppler flow studies was ordered, but not completed since the patient’s respiratory status rapidly deteriorated and he had to be intubated. After intubation, the echo was obtained and although it showed no major structural defects of the heart, it did help diagnose the patient with pulmonary hypertension. Although there is no cure for pulmonary hypertension, the prognosis for this patient is good since the disease was caught early. Prognosis depends on how well the patient responds to treatment and whether or not he has signs of heart failure.
Treatment
Once the diagnosis of pulmonary hypertension was made, the patient was placed on sildenafil IV q6hrs. Nitric oxide (NO) at 20ppm was added to the ventilator to aid in pulmonary dilation and oxygenation. In addition to following daily CBC’s, chemistry, and ABG’s, methemoglobin levels were followed closely while the patient was on NO.
Outcome
After several weeks on the ventilator, the patient was trached and slowly weaned to minimal ventilator settings. The NO was weaned slowly, sildenafil was changed to PO and the patient was transferred to a long-term rehabilitation center, where he continues to do well.