Persistent pulmonary hypertension of the newborn

Case contributed by Roy Waknin
Diagnosis certain

Presentation

A baby girl at 36 weeks and 1 day gestational age was delivered under general anesthesia. She was intubated in the delivery room due to hypoxemia and no heart rate at 1 and 5 minutes.

Patient Data

Age: 0 days
Gender: Female

4 hours after birth

x-ray

Moderately severe cardiomegaly with moderate pulmonary vascular congestion and mild interstitial edema due to cardiac etiology or a shunt.

There is an endotracheal tube, orogastric tube, umbilical vein catheter, and umbilical artery catheter placed.

Given the radiographic findings, an echocardiogram was performed to investigate the etiology of the cardiomegaly and pulmonary vascular congestion.

Conclusions found:

  • patent foramen ovale (PFO) present with right to left shunt
  • normal cardiac anatomy with systemic right ventricle (RV) pressures
  • moderate pulmonary and tricuspid valve insufficiency
  • mild mitral regurgitation
  • moderately depressed RV function

The diagnosis of persistent pulmonary hypertension of the newborn (PPHN) was confirmed and the baby girl was treated accordingly.

1 day after birth following Rx

x-ray

Overall improving bilateral lung aeration and interstitial edema in this baby now being treated for PPHN with surfactant and iNO.

Stable moderately severe cardiomegaly and improving moderate central pulmonary vascular congestion.

The endotracheal tube, orogastric tube, umbilical vein catheter, and umbilical artery catheter are still present.

Case Discussion

This baby girl presented upon delivery in cardiorespiratory distress and was immediately intubated. Radiographic imaging of the chest revealed cardiomegaly, pulmonary vascular congestion, and interstitial pulmonary edema, all of which are signs of a cardiac etiology or the presence of a shunt. 

After echocardiogram was performed and confirmed a patent foramen ovale (PFO) causing a persistent shunt, the diagnosis of persistent pulmonary hypertension of the newborn (PPHN) was confirmed and treatment with surfactant and inhaled nitric oxide was started.

Follow-up radiographic imaging the following day showed early signs of improvement with decreased pulmonary edema and vascular congestion.

Another echocardiogram was performed to evaluate cardiac changes, and it found:

  • PFO is still present however shunt is now left to right instead of right to left
  • There is a patent ductus arteriosus (PDA) with a left to right shunt
  • Right ventricle (RV) size and function have improved to normal

The treatment for PPHN was continued until the PFO and PDA closed.

 

This case was submitted with supervision and input from:

Soni C. Chawla, M.D.                                                                                                
Associate Professor                                       
Department of Radiological Sciences                      
David Geffen School of medicine at UCLA               
Olive View-UCLA Medical Center

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