Assessment of the Reversibility of Pulmonary Hypertension in Adult Congenital Heart Defects: When, how and why?

Authors

  • D. Echazarreta Head of Cardiology Room, San Juan de Dios University Hospital, La Plata, Buenos Aires, 27 and 70 Street La Plata, Buenos Aires, CP 1900, Argentina
  • L. Ortíz Adult Congenital Heart Disease Section, San Juan de Dios University Hospital, La Plata, Buenos Aires, 27 and 70 Street La Plata, Buenos Aires, CP 1900, Argentina
  • M. Maydana Heart Failure and Pulmonary Hypertension Section, San Juan de Dios University Hospital, La Plata, Buenos Aires, 27 and 70 Street La Plata, Buenos Aires, CP 1900, Argentina
  • M. Gomez Nuclear Medicine Service Staff, San Juan de Dios University Hospital, La Plata, Buenos Aires, 27 and 70 Street La Plata, Buenos Aires, CP 1900, Argentina
  • A. Hauqui Staff of Hemodynamic Service, San Juan de Dios University Hospital, La Plata, Buenos Aires, 27 and 70 Street La Plata, Buenos Aires, CP 1900, Argentina
  • I. Rifourca Head of Hemodynamic Service, San Juan de Dios University Hospital, La Plata, Buenos Aires, 27 and 70 Street La Plata, Buenos Aires, CP 1900, Argentina
  • M. Portis Head of Cardiology Service, San Juan de Dios University Hospital, La Plata, Buenos Aires, 27 and 70 Street La Plata, Buenos Aires, CP 1900, Argentina

DOI:

https://doi.org/10.12970/2311-052X.2020.08.02

Keywords:

 Buccal flap, systematic review, perceptual speech assessment, speech outcomes, surgical outcomes.

Abstract

Pulmonary arterial hypertension (PAH) in the context of adult congenital heart disease (ACHD) can be reversed by early closure of the communication or pre-existing shunt. This "window of opportunity" is lost beyond a certain point of no return. Therefore, it is crucial to accurately assess the reversibility of this progressive vascular disease, which usually begins in early stages. The reversibility assessment is currently based on a combination of clinical symptoms, hemodynamic changes and fundamental variables such as pulmonary vascular resistance. However, its measurement has limited predictive value and leaves many patients in a “gray area” regarding decision-making. This review provides a concise overview of the mechanisms involved in the flow-dependent progression of PAH in CHD and assesses existing and future alternatives for a thorough assessment of existing pulmonary artery disease. The structural quantification of the pulmonary arterial tree using fractal branching algorithms, functional images with intravascular ultrasound, nuclear imaging, new serum biomarkers, genetic tests and the potential for transcriptomic analysis of circulating endothelial cells and platelets are being incorporated into the evaluation of this type of patients. Keywords: Pulmonary Hypertension-Congenital Heart Diseases-Pulmonary Vasculature-Heart Failure.

References

van Dissel AC, Mulder BJ, Bouma BJ. The Changing Landscape of Pulmonary Arterial Hypertension in the Adult with Congenital Heart Disease. J Clin Med 2017; 6(4). https://doi.org/10.3390/jcm6040040

Xu ZY, et al. [Risk factors for death and the clinical features of different subtypes of patients with pulmonary arterial hypertension related to congenital heart disease]. Zhonghua Xin Xue Guan Bing Za Zhi 2020; 48(4): 315-322.

Deng X, et al. Guideline implementation and early risk assessment in pulmonary arterial hypertension associated with congenital heart disease: A retrospective cohort study. Clin Respir J 2019; 13(11): 693-699. https://doi.org/10.1111/crj.13076

Lammers AE, et al. Pulmonary hypertension after shunt closure in patients with simple congenital heart defects. Int J Cardiol 2020; 308: 28-32. https://doi.org/10.1016/j.ijcard.2019.12.070

Hasan B, et al. Challenges and Special Aspects of Pulmonary Hypertension in Middle- to Low-Income Regions: JACC State-of-the-Art Review. J Am Coll Cardiol 2020; 75(19): 2463-2477. https://doi.org/10.1016/j.jacc.2020.03.047

Kuang HY, et al. The efficiency of endothelin receptor antagonist bosentan for pulmonary arterial hypertension associated with congenital heart disease: A systematic review and meta-analysis. Medicine (Baltimore) 2018; 97(10): e0075. https://doi.org/10.1097/MD.0000000000010075

Lopes AA, et al. 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). Pulm Circ 2014; 4(2): 330-41. https://doi.org/10.1086/675995

Galie N, et al. An overview of the 6th World Symposium on Pulmonary Hypertension. Eur Respir J 2019; 53(1). https://doi.org/10.1183/13993003.02148-2018

Galie N, Simonneau G. The Fifth World Symposium on Pulmonary Hypertension. J Am Coll Cardiol 2013; 62(25 Suppl): D1-3. https://doi.org/10.1016/j.jacc.2013.10.030

Al-Omary MS, et al. Pulmonary Hypertension Due to Left Heart Disease: Diagnosis, Pathophysiology, and Therapy. Hypertension 2020; 75(6): 1397-1408. https://doi.org/10.1161/HYPERTENSIONAHA.119.14330

Hsu S, et al. Multi-Beat Right Ventricular-Arterial Coupling Predicts Clinical Worsening in Pulmonary Arterial Hypertension. J Am Heart Assoc 2020; e016031. https://doi.org/10.1161/JAHA.119.016031

Desai A, et al. Dual-Energy Computed Tomography as an Alternative Noninvasive Study for Evaluation of Chronic Thromboembolic Pulmonary Hypertension Postoperatively. Circ Cardiovasc Imaging 2020; 13(5): e010168. https://doi.org/10.1161/CIRCIMAGING.119.010168

Yu YZ, et al. Changed hemodynamics in acute vasoreactivity testing: prognostic predictors in chronic thromboembolic pulmonary hypertension. Am J Transl Res 2020; 12(3): 959-973.

Harbaum L, et al. The application of 'omics' to pulmonary arterial hypertension. Br J Pharmacol 2020. https://doi.org/10.1111/bph.15056

Padervinskiene L, et al. Identification of Cardiac MRI and Bio-Marker Thresholds for One-Year Survival in Pre-Capillary Pulmonary Hypertension: Prospective Study. Medicina (Kaunas) 2020; 56(4). https://doi.org/10.3390/medicina56040167

Wang L, et al. Assessment of lung glucose uptake in patients with systemic lupus erythematosus pulmonary arterial hypertension: a quantitative FDG-PET imaging study. Ann Nucl Med 2020. https://doi.org/10.1007/s12149-020-01461-y

Marsboom G, et al. Lung (1)(8)F-fluorodeoxyglucose positron emission tomography for diagnosis and monitoring of pulmonary arterial hypertension. Am J Respir Crit Care Med 2012; 185(6): 670-9. https://doi.org/10.1164/rccm.201108-1562OC

Gebka A, et al. Serum N-terminal pro-B-type natriuretic peptide levels are associated with left atrial dilation, resting left ventricular outflow tract gradient, and pulmonary hypertension in patients with hypertrophic cardiomyopathy. Postepy Kardiol Interwencyjnej 2020; 16(1): 97-101. https://doi.org/10.5114/aic.2019.91516

Miao H, et al. Novel angiogenesis strategy to ameliorate pulmonary hypertension. J Thorac Cardiovasc Surg 2020. https://doi.org/10.1016/j.jtcvs.2020.03.044

Mathew R, et al. Pulmonary Hypertension Remodels the Genomic Fabrics of Major Functional Pathways. Genes (Basel) 2020; 11(2). https://doi.org/10.3390/genes11020126

Chen C, et al. Metabolomics reveals metabolite changes of patients with pulmonary arterial hypertension in China. J Cell Mol Med 2020; 24(4): 2484-2496. https://doi.org/10.1111/jcmm.14937

Elinoff JM, et al. Meta-analysis of blood genome-wide expression profiling studies in pulmonary arterial hypertension. Am J Physiol Lung Cell Mol Physiol 2020; 318(1): L98-L111. https://doi.org/10.1152/ajplung.00252.2019

Welch CL, Chung WK. Genetics and Other Omics in Pediatric Pulmonary Arterial Hypertension. Chest 2020; 157(5): 1287-1295. https://doi.org/10.1016/j.chest.2020.01.013

Brida M, et al. Cardiac catheter intervention complexity and safety outcomes in adult congenital heart disease. Heart 2020. https://doi.org/10.1136/heartjnl-2019-316148

Downloads

Published

2020-04-20

Issue

Vol. 8 (2020)

Section

Articles