Unique Pulmonary Hypertension in Young Children: A Case Series Study

Abstract

Pediatric pulmonary hypertension (PH) has a similar clinical presentation to the adult disease but is associated with several additional disorders and challenges that require a specific approach for their fulminant course. With improved care for premature infants, various forms of pulmonary vascular disease have been found in children that did not previously exist. Pediatric PH can begin in utero, resulting in pulmonary vascularity growth abnormalities that may persist into adulthood. Here, we retrospectively reviewed several unique pediatric PH cases from 2000 to 2020 at Kaohsiung Medical University Hospital, Taiwan, a tertiary teaching hospital. Their comorbidities varied and included surfactant dysfunction, bronchopulmonary dysplasia, premature closure of the ductus arteriosus, high levels of renin and aldosterone, and Swyer-James-Macleod syndrome. Their clinical profiles, radiological characteristics, echocardiography, pulmonary angiogram, and therapeutic regimens were recorded. Further, because the underlying causes of pediatric PH were complex and markedly different according to age, adult PH classification may not be applicable to pediatric PH in all settings. We also classified these cases using different systems, including the Panama classification and the Sixth World Symposium on PH, and compared their advantages and disadvantages.

Keywords: Swyer–James–Macleod syndrome; bronchopulmonary dysplasia; children; pulmonary hypertension; renin; taurine.

Figure 1

(A) Chest radiography of a male neonate presenting with cyanosis and pulmonary hypertension, revealing diffuse ground glass lungs with low volumes; (B) lung section showing that the alveolar ducts, alveoli, and terminal and respiratory bronchioles are lined with an eosinophilic hyaline membrane with necrotic alveolar lining cells and fibrin (40×).

Figure 2

Chest radiography of a 52-day-old full-term male infant presenting with cyanosis and diagnosed with pulmonary hypertension showed bilateral hyperlucent lung and cardiomegaly with a cardiothoracic ratio of 0.64.

Figure 3

An extremely preterm male infant was diagnosed with bronchopulmonary dysplasia (BPD) and pulmonary hypertension. Chest radiography at the age of 3 years demonstrated dextrocardia, widespread coarse interstitial markings, atelectasis, and regions of hyperexpansion (A). A preterm female infant was diagnosed with BPD and pulmonary hypertension. Her chest radiography at the age of 2 years also showed the coarse interstitial markings, atelectasis, and regions of hyperexpansion (B) typical of BPD.

Figure 4

A 3-month-old female infant was diagnosed with pulmonary hypertension with high levels of renin and aldosterone syndrome. Chest radiography showed a mild ground glass appearance and cardiomegaly with a cardiothoracic ratio of 0.75 (A). Long-axis echocardiographic images (two-dimensional (B) and M-mode (C)) demonstrated a hypertrophic right ventricle, which was much improved 6 months after candesartan (angiotensin receptor blocker) and captopril (angiotensin-converting enzyme inhibitor) were given (D,E).

Figure 5

Long-axis echocardiographic image in a 1-day-old female newborn revealed a hypertrophic right ventricle without patent ductus arteriosus noted (A), which improved 5 days later (B).

Figure 6

A 3-year-old boy presenting with hemoptysis and dyspnea was diagnosed with Swyer–James–Macleod syndrome and pulmonary hypertension. Chest radiography revealed a unilateral hyperlucent lung (A); a bronchoscopy revealed several erosions and bleeding from the trachea (arrow) (B); coronal-view high-resolution computed tomography of the chest revealed regional ground-glass opacities and focal emphysema, particularly in the left upper lobe (C); dual-energy computer tomography revealed areas of decreased lung attenuation, associated with decreased pulmonary blood vasculature in the left lung (D); perfusion scintigraphy revealed marked defects in left lung fields (E); and ventilation scintigraphy revealed a marked defect corresponding to the previous lab tests (F). C, carina; R, right side; L, left side.