Repair of Congenital Heart Disease with Associated Pulmonary Hypertension in Children: What are the Minimal Investigative Procedures? Consensus Statement from the Congenital Heart Disease and Pediatric Task Forces, Pulmonary Vascular Research Institute (PVRI)

Abstract

Standardization of the diagnostic routine for children with congenital heart disease associated with pulmonary arterial hypertension (PAH-CHD) is crucial, in particular since inappropriate assignment to repair of the cardiac lesions (e.g., surgical repair in patients with elevated pulmonary vascular resistance) may be detrimental and associated with poor outcomes. Thus, members of the Congenital Heart Disease and Pediatric Task Forces of the Pulmonary Vascular Research Institute decided to conduct a survey aimed at collecting expert opinion from different institutions in several countries, covering many aspects of the management of PAH-CHD, from clinical recognition to noninvasive and invasive diagnostic procedures and immediate postoperative support. In privileged communities, the vast majority of children with congenital cardiac shunts are now treated early in life, on the basis of noninvasive diagnostic evaluation, and have an uneventful postoperative course, with no residual PAH. However, a small percentage of patients (older at presentation, with extracardiac syndromes or absence of clinical features of increased pulmonary blood flow, thus suggesting elevated pulmonary vascular resistance) remain at a higher risk of complications and unfavorable outcomes. These patients need a more sophisticated diagnostic approach, including invasive procedures. The authors emphasize that decision making regarding operability is based not only on cardiac catheterization data but also on the complete diagnostic picture, which includes the clinical history, physical examination, and all aspects of noninvasive evaluation.

Hemodynamic assessment and acute pulmonary vasoreactivity testing in the evaluation of children with pulmonary vascular disease. Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK

“…Catheter assessment of cardiovascular haemodynamics and acute vasoreactivity testing (AVT) in the catheterisation laboratory remain the gold standard for the diagnosis and prognostication of PH 2–4. Invasive assessment of PH follows a non-invasive diagnostic workup and has to be performed in the context of the patient's age, medical history and functional status 5. A systematic catheterisation protocol is required and has been established in a very standardised manner for the adult population, however, for paediatric PH it usually varies among expert referral centres 6.…”

Pulmonary hypertension in children with congenital heart disease (PAH-CHD, PPHVD-CHD). Expert consensus statement on the diagnosis and treatment of paediatric pulmonary hypertension. The European Paediatric Pulmonary Vascular Disease Network, endorsed by ISHLT and DGPK

“…Algorithm for the management of patients with congenital heart disease associated with PAH/PPVD and congenital shunt lesions (modified from Lopes et al 13 and the PVRI PAH-CHD taskforce (pdf on PVRI website). The indication for invasive diagnoses and eligibility for surgery/operability by comprehensive left and right heart catheterisation includes basic evaluation and AVT, the latter especially in the grey zone of forecast uncertainty.…”

Approaching atrial septal defects in pulmonary hypertension

“…Guidelines for evaluation of operability in CHD with pulmonary hypertension do not offer clear cutoff values [14,15]. In the very young patient, one is usually confronted with a high pulmonary flow condition with low PVR.…”

“…The procedure has bleeding risks, and the accurate histological interpretation of the biopsy requires special expertise, not widely available anymore. However, lung biopsy can still provide helpful information in very selected cases and is also still used for research purposes [15]. Studies with lung biopsies led to the current concept of how shunt-induced PAH evolves.…”

“…A PVR cutoff that precludes defect closure in the absence of an Eisenmenger syndrome has not been established [14,15]. In the study of Steele et al including 26 adults with PAH (defined as total pulmonary resistance >7 WU * m 2 ) undergoing cardiac surgery, 22 had a total pulmonary resistance <15 WU * m 2 and 19 of them had a significant improvement during the long-term follow-up (median follow-up, 12 years), whereas all four adults with a total pulmonary resistance ‡15 WU * m 2 prior to surgery died during follow-up [19].…”