5.4 PPHN, Pulmonary Hemorrhage, Pleural Disease, and Infection
Key Takeaways
- PPHN treatment is a stack: oxygen, optimized lung volume, correction of acidosis hypothermia and hypoglycemia, inotropes so systemic pressure can compete with pulmonary pressure, iNO once the lung is recruited, HFOV when atelectasis or MAS heterogeneity blocks gas exchange, and ECMO when the oxygenation index remains extreme on maximal therapy and disease is potentially reversible.
- Typical iNO start is 20 ppm with methemoglobin monitoring and no abrupt stops. OI = (MAP × FiO2 × 100) / PaO2; a historical ECMO conversation often begins around OI >40, with center-specific criteria.
- Pulmonary hemorrhage is sudden blood from the ETT, often after surfactant in an infant with a flooding PDA. Increase mean airway pressure to tamponade, replace blood, treat coagulopathy, and address the duct—do not dismiss it as suction trauma.
- Pneumothorax needs transillumination, needle aspiration for tension, and a chest tube for ongoing leak. Hemothorax, empyema, and effusion are blood, pus, and fluid versions of the same occupied pleural space.
- Pneumonia and sepsis may be bacterial, viral (including RSV conceptually), or fungal. Ureaplasma is associated with BPD in observational work; association is not dogma that every colonized infant has pneumonia that antibiotics will erase.
PPHN, Pulmonary Hemorrhage, Pleural Disease, and Infection
Quick Answer: PPHN is extra-uterine pulmonary vascular resistance that stays fetal, with right-to-left shunting and labile hypoxemia. Treatment is oxygen, optimized lung volume, inhaled nitric oxide (iNO) when the lung is recruited, inotropes to keep systemic pressure competitive, HFOV when atelectasis or MAS heterogeneity blocks iNO delivery, and ECMO when the oxygenation index remains extreme on maximal therapy and the lung disease is potentially reversible. Pulmonary hemorrhage is often a PDA-flood plus coagulopathy event; increase mean airway pressure, replace blood, and treat the duct. Pneumothorax, hemothorax, empyema, and pleural effusion need recognition and drainage. Infection is bacterial, viral, and fungal; Ureaplasma is an association with chronic lung disease, not a dogma that every colonized infant has pneumonia you must treat the same way.
Pulmonary hypertension, pulmonary hemorrhage, pleural-space abnormalities, and infection are listed respiratory leaves on the current Neonatal CCRN Test Plan. Cardiovascular teaching already owned the shunt mechanism (pre- versus post-ductal saturations, failed PVR drop). This OpenExamPrep section owns the management stack and the neighboring bleeding, pleural, and infectious crises. Full ECMO circuit plumbing belongs with later advanced-therapy teaching; here you need indications and the call.
PPHN management in an ordered stack
Incidence is often quoted near 2 per 1000 live births. Mechanisms: maladaptation (MAS, asphyxia, sepsis—structurally normal but reactive vessels), maldevelopment (chronic fetal hypoxia, premature constriction of the ductus in utero), and underdevelopment (CDH, pulmonary hypoplasia, oligohydramnios sequence).
Diagnosis reminders: right-hand versus foot saturations, loud S2, lability, and an echocardiogram that shows right-to-left shunt and estimated high right-ventricular pressure. The hyperoxia test overlaps with cyanotic heart disease; PPHN may raise PaO2 less than pure lung disease and more than transposition with poor mixing. Echo is the sorter. A term infant with MAS, a right-hand SpO2 of 92% and a foot SpO2 of 80% on 80% oxygen is telling you ductal right-to-left flow.
Oxygenation index (OI) = (MAP × FiO2 × 100) / PaO2, with FiO2 as a fraction (0.80, not 80). Example: MAP 16, FiO2 1.0, PaO2 40 mm Hg → OI = 40. Many ECMO conversations begin when OI is in the 20s–40s despite optimized care; a classic historical threshold for ECMO consideration is OI >40. Centers differ. Know the formula and that a rising OI means the infant is losing, not that you should wait for a round number without calling the ECMO hospital.
Stack, in the order you can actually nurse:
- Lung volume. iNO cannot vasodilate units that are collapsed or full of meconium. Recruit with PEEP, CPAP, or HFOV. Avoid overdistention that raises PVR by compressing extra-alveolar vessels.
- Oxygen. Hypoxic pulmonary vasoconstriction is the fetal reflex you are trying to turn off. Use oxygen to hit the unit’s saturation target (often pre-ductal ≥95% in acute PPHN pathways—follow written orders; this is not an HLHS 75–85% target).
- Metabolic hygiene. Core temperature 36.5–37.5 °C. Treat hypoglycemia. Correct severe acidosis while restoring perfusion—pH <7.25 keeps vessels tight. Quiet-term heart rate remains about 120–160; a PPHN infant at 190 who is cold and 35.8 °C is not “just irritable.”
- Inotropes and vasopressors. If SVR falls below PVR, blood goes right to left. Dopamine, epinephrine, vasopressin, and milrinone appear in protocols. Milrinone drops PVR and SVR; have a pressure plan. This chapter does not publish a secret AACN mcg/kg/min card.
- iNO. Typical start is 20 ppm. Monitor methemoglobin and nitrogen dioxide. Never stop iNO abruptly (rebound pulmonary hypertension). If OI does not improve, ask whether the lung is recruited and whether the diagnosis is actually cyanotic congenital heart disease.
- HFOV. MAS, pneumonia, and CDH hypoplasia often need a stable MAP rather than large conventional tidal swings.
- Sedation and paralysis in selected labile infants to stop the desaturation-crying spiral—trade-off is awareness of IVH and edema in preterms.
- ECMO when medical therapy fails, disease is reversible, and the infant meets center criteria (historically often ≥34 weeks and ≥2000 g, no lethal malformation, no uncorrectable coagulopathy—confirm locally).
Worked example: a 39-week MAS infant on conventional ventilation, MAP 14, FiO2 1.0, PaO2 45 (OI 31) still has a 12-point pre/post gap. The film is white and under-recruited. Starting iNO without raising lung volume is the wrong first leap. Recruit, then iNO. If OI climbs through 40 on HFOV plus 20 ppm iNO plus blood pressure support, that is an ECMO call, not another hour of hoping.
Pulmonary hemorrhage
Sudden bright blood from the ETT, a white-out radiograph, and a crash in compliance define pulmonary hemorrhage. Classic setup: an extremely preterm infant a few days old, a large left-to-right PDA, recent surfactant that dropped PVR, and a lung that suddenly flooded until capillaries rupture. Asphyxia, coagulopathy, and volume overload contribute. Heart rate may spike to 180–200, saturations fall into the 50s–60s, and the ventilator alarms for low tidal volume.
Immediate moves: call for help, increase PEEP/MAP to tamponade the leak (often a modest increase—follow protocol, not a random jump to 20 cm H2O), endotracheal epinephrine is used in some hemorrhage algorithms, blood products for volume and coagulopathy, and echocardiography for the duct. Surfactant may be repeated after the hemorrhage because blood inactivates it. Do not treat this as “a little bloody secretions from suction trauma” if the saturation is 40% and the ventilator is shrieking.
Worked example: a 26-week infant, day 3, just received surfactant. Pulses are bounding, blood pressure is 55/18 mm Hg, the liver is down, and pink froth fills the ETT. That is PDA-associated hemorrhage physiology until the echo says otherwise.
Pleural space: air, blood, pus, fluid
Pneumothorax: transillumination in a small infant, shifted heart tones, unequal breath sounds, sudden bradycardia. Tension physiology is obstructive shock. Needle aspiration (commonly the second intercostal space, midclavicular line, or a lateral site per unit) is the emergency; a chest tube is the durable drain. After MAS, PIE, or high MAP, assume air leak until proven otherwise. A term MAS infant who was 90% and is now 50% with a shifted point of maximal impulse is not “needs more iNO first.”
Hemothorax: blood in the pleura after surgery, trauma, or a vascular accident. Volume loss plus a white hemithorax. Drain and replace blood; this is not a diuretic problem.
Pleural effusion: hydrops, heart failure, lymphatic disorders, hypoalbuminemia. Drainage if the lung cannot expand. Output is charted in mL/kg.
Empyema: infected pleural fluid, usually a complication of bacterial pneumonia. Antibiotics plus drainage; loculations may need surgery. The infant is febrile, the effusion is not simple straw fluid, and inflammatory markers stay high.
Chest-tube nursing: underwater seal, oscillation with respiration, no dependent loops full of fluid, and never clamp a bubbling tube because you wanted a tidy bedspace. A sudden quiet tube plus a falling blood pressure after sternotomy or chest surgery is clot or tamponade physiology until proven otherwise.
| Occupant | Typical neonate | First durable move |
|---|---|---|
| Air (pneumothorax) | MAS, PIE, high MAP, after resuscitation | Needle if tension; chest tube for ongoing leak |
| Blood (hemothorax) | Postoperative, trauma | Drain plus volume and blood products |
| Pus (empyema) | Bacterial pneumonia that loculates | Antibiotics plus drainage |
| Fluid (effusion/chyle) | Hydrops, heart failure, lymphatic leak | Drain; treat cause; MCT or TPN if chyle |
Infection: bacterial, viral, fungal—and Ureaplasma without dogma
Bacterial pneumonia and sepsis in the first 72 hours is early-onset territory (GBS, E. coli, other enteric organisms). The radiograph overlaps RDS. Treat empirically when the infant is ill; do not wait for a perfect film. Late-onset pneumonia is often ventilator-associated or hematogenous (Staphylococcus aureus, coagulase-negative staphylococci as a line story, gram-negatives). Isolation, cultures, and timely antibiotics are the nursing-visible parts; the full early- versus late-onset sepsis algorithm is expanded in the multisystem sepsis chapter.
Viral: HSV can present as pneumonitis in a systemic picture—think vesicles, seizures, and term or preterm collapse, and treat while you test. CMV is more a congenital or chronic story. RSV is a seasonal small-airway disaster in former preterms with BPD: apnea, wheeze, hyperinflation, isolation, and supportive oxygen. Palivizumab is a prevention product for selected high-risk infants per seasonal guidance—it is not a treatment for established RSV bronchiolitis. Avoid a reflex antibiotic course unless bacterial superinfection is likely.
Fungal: Candida in the extreme preterm with central lines and broad-spectrum antibiotics can seed the lung as part of disseminated disease. Amphotericin or fluconazole pathways are protocol-driven. A 24-week infant on day 18 with a new infiltrate, thrombocytopenia, and a long-dwelling central line is not “only RSV season.”
Ureaplasma (and related genital mycoplasmas) colonizes the preterm airway and is associated with BPD/CLD in observational work. Association is not proof that every positive PCR is the cause of that infant’s oxygen need, and it is not proof that antibiotics will erase BPD. Mention it as a risk association, not as a mandatory treat-all dogma. If your unit treats selected colonized, high-risk extremely preterm infants, that is a protocol decision—not a CCRN requirement to start a drug you cannot name from an unpublished handbook.
Exam traps: giving iNO to an unrecruited lung; using HLHS saturation targets in PPHN; calling every ETT blood trauma; clamping a bubbling chest tube; treating RSV with routine antibiotics; and claiming Ureaplasma colonization always equals pneumonia.
Independent mixed-item practice is at /practice/ccrn-neonatal. Pediatric PPHN-style pulmonary hypertension in older children is a different time course; this stack is neonatal.
A 40-week infant with MAS is on conventional ventilation, MAP 12 cm H2O, FiO2 1.0, PaO2 40 mm Hg, with a white under-recruited radiograph and a 10-point pre/post-ductal saturation gap. Which next-step idea is most accurate?
A 25-week infant on day 3 receives surfactant. One hour later, bounding pulses, a blood pressure of 52/16 mm Hg, and bright blood fill the endotracheal tube; saturations fall to 55%. Which mechanism and first support idea match?
A term MAS infant suddenly bradycardias to 70, saturations fall to 48%, the left chest transilluminates, and heart tones shift right. What is the priority intervention concept?
Which statement about neonatal respiratory infection is accurate?