Parameters associated with outcome in pediatric patients with congenital heart disease and pulmonary hypertension subjected to combined vasodilator and surgical treatments

Abstract

Management of pediatric pulmonary hypertension associated with congenital heart disease (PHT-CHD) is challenging. Some patients have persistently elevated pulmonary artery pressure (PAP) after cardiac surgery, an undesired condition that is difficult to predict. We investigated the value of clinical, hemodynamic, and histopathological data in predicting the outcome in a prospective cohort. Patients with PHT-CHD received sildenafil orally pre- and postoperatively for six months and then were subjected to a catheter study. Thirty-three patients were enrolled (age range = 4.6–37.0 months). Pulmonary vascular resistance (PVR) was 4.9 (range = 3.9–7.2) Wood units × m2 (median with IQR). Twenty-two patients had a ≥ 20% decrease in PVR and pulmonary-to-systemic vascular resistance ratio (PVR/SVR) in response to inhaled nitric oxide (NO). The response was directly related to the degree of medial hypertrophy of pulmonary arterioles ( P < 0.05) (morphometric analysis, intraoperative lung biopsy). Subsequently, five of the non-responders had a ≥ 30% increase in pulmonary blood flow in response to sildenafil (3.0 [2.0–4.0] mg/kg/day). Six months after surgery, PAP and PVR were significantly lower ( P < 0.001 vs. baseline), even in seven patients with Heath-Edwards grade III/IV pulmonary vascular lesions ( P = 0.018), but still abnormal in 12 individuals (>25 mmHg and >3.0 U × m2, respectively). A preoperative PVR/SVR of ≥24% during NO inhalation and a wall thickness of arteries accompanying respiratory bronchioli of ≥4.7 (Z score) were identified, respectively, as risk and protection factors for abnormal postoperative hemodynamics (hazard ratio [95% CI] = 1.09 [1.01–1.18], P = 0.036; and 0.69 [0.49–0.98], P = 0.040, respectively). Thus, in PHT-CHD patients receiving oral sildenafil pre- and post-surgical repair of cardiac lesions, mid-term postoperative outcome is predictable to some extent.

Survival of Left-to-Right Shunt Repair in Children with Pulmonary Arterial Hypertension at a Tertiary Hospital in a Low-to-Middle-Income Country

“…From the restricted mean survival time regression analysis, we found that PVRi was a prognostic factor for the difference of survival. To our knowledge, these two parameters had been shown to be predictors of mortality in PAH-CHD [ 15 23 25 ].…”

“…These echocardiography parameters have not been validated and compared with the catheterization criteria henceforth making a discrepancy data especially between mPAP and PVRi. Although, there is still a possibility of reduced baseline PVR due to the effects of general anesthesia and the use of the oxygen fraction prior to hemodynamic measurements during cardiac catheterization [ 25 38 ]. These might have resulted in reduced baseline PVRi in our study.…”

“Treat and repair” strategy for shunt lesions: a critical review

“…In a recently published series from Brazil, 33 patients with pre-defined high-risk features (presence of at least three of the five following criteria: absence of clinical features of pulmonary overcirculation, bidirectional shunt across the defect, sustained or intermittent systemic desaturation <90%, Down syndrome, age >18 months) were administered sildenafil for a mean duration of just 27 days before undergoing surgery. 39 The median age of the population was only 9.7 months, and median baseline PVRI was 4.5 WU.m 2 . Although the authors concluded that the midterm postoperative outcomes are predictable to an extent in patients receiving sildenafil pre- and post-surgery, the young age, very short duration of therapy, mildly elevated PVRI and high prevalence of Down syndrome in the study population make this conclusion less generalizable.…”

Pulmonary Hypertension in Children across Africa: The Silent Threat

“…Monotherapy with phosphodiesterase 5 inhibitors (PDE 5i) is recommended for low-risk PH [ 2 , 70 ] such as CHD postoperative with residual PAH and persistent pulmonary hypertension of the newborn [ 2 ]. ECMO has been recommended as a treatment option augmented with sildenafil and prostacyclin for refractory disease [ 3 ].…”

“…Different schools of thought offer scenarios including the presence of left to right shunt, oxygen saturations above 93-95%, and age below 9 months-2 years, as well as clinical acumen based on a holistic agreement from heart team discussions as basic criteria for assessing operability [ 5 , 30 , 70 , 77 ]. Oxygen saturation below 90% is predictive of inoperability, and for children with PDAs, oxygen saturations should be taken from the lower limbs [ 30 , 57 ].…”