Abstract
Separately for each of the subgroups of pulmonary hypertension associated with congenital heart disease, characteristic clinical symptoms and physical examination data are considered. An increase in pulmonary vascular resistance after radical correction and with small / coincidental defects leads to insufficient filling of the systemic ventricle and the progression of symptoms of low cardiac output – shortness of breath, palpitations, fatigue, weakness and decreased exercise tolerance. In the later stages, due to transient systemic hypotension, dizziness, lightheadedness and fainting are added, as well as signs of congestive right ventricular failure – peripheral edema, liver enlargement, ascites, and sometimes neck vein swelling. In Eisenmenger syndrome, cyanosis and signs of its systemic complications are observed – polycythemia, deformation of the distal phalanges of the fingers like "drumsticks" and "watch glasses", posture disturbance due to osteoarthropathy and scoliosis, pulmonary and paradoxical systemic thrombosis and embolism, bleeding, symptoms of gout and cholelithiasis , impaired renal function. Already mild pulmonary hypertension after Fontaine's operation causes venous hypertension with congestive heart failure, hydrothorax, protein deficiency enteropathy and plastic bronchitis, as well as insufficient blood flow to the systemic ventricle with low cardiac output, shortness of breath, palpitations, fatigue, weakness and decreased physical activity. In all forms of pulmonary hypertension, pain in the region of the heart, hemoptysis, pulmonary hemorrhage, arrhythmias, and developmental delay in children may occur. Inspection must be carried out "from head to toe." The diagnostic value of auscultation depends on the complexity of the congenital malformation leading to pulmonary hypertension. Pulse oximetry should be performed separately on the arms and legs at rest and exercise.
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About the authors
- Sergey V. Gorbachevsky, Dr. Med. Sci., Professor, Head of Department; ORCID
- Anton A. Shmalts, Dr. Med. Sci., Leading Research Associate, Associate Professor; ORCID
