Author + information
- Rogerio Souz, MD, PhD* ( and )
- Gerald Simonneau, MD, PhD
- ↵*Pulmonary Department, Heart Institute, University of Sao Paulo Medical School, R. Afonso de Freitas 421-112, Sao Paulo, SP04006052, Brazil
The relevance of congenital heart disease (CHD) in the setting of pulmonary arterial hypertension (PAH) is unquestionable, as stated in the letter by Dr. Kothari. However, it is important to emphasize that the core structure of the updated clinical classification of pulmonary hypertension (1) is not based on the relevance of the topic or on the prevalence of the disease; thus, there is no hierarchical level within each one of the groups. The fact that schistosomiasis-associated PAH is classified as item 1.4.5 does not make it more or less important than human immunodeficiency virus infection–associated PAH, classified as item 1.4.2. The same can be directly extrapolated to the other 4 groups. Consequently, one could not consider that CHD-associated PAH has been downgraded from the previous classification. This classification has even strengthened the role of CHD by describing not only the anatomic-pathophysiologic classification of congenital systemic-to-pulmonary shunts associated with PAH (see Table 4 in the classification ) but also the clinical classification of congenital systemic-to-pulmonary shunts associated with PAH (see Table 5 in the classification ) to disseminate the current knowledge on the management of such relevant conditions.
The prevalence of the different forms of pulmonary hypertension is extremely important for the appropriate understanding of the whole pulmonary hypertension scenario, mainly considering that regional characteristics, such as local altitude, sanitary conditions, and health care infrastructure and organization, may play a significant role (2). Even considering that this is not related to the order in the updated classification, the commentary from Dr. Kothari should be considered as a reinforcement of the need for robust registries of the different forms of pulmonary hypertension (3) to serve as a basis for international collaborative efforts in the pulmonary hypertension field.
- American College of Cardiology Foundation