Pulmonary artery hypertension (PAH) is characterized by remodelling of the small pulmonary arteries, leading to a progressive increase in pulmonary vascular resistance and right ventricular failure. The clinical classification of PH is intended to categorize multiple clinical conditions into five groups according to their similar clinical presentation, pathological findings, hemodynamic characteristics, and treatment strategy as idiopathic or associated PAH (group 1), PH due to left heart disease (group 2), PH due to lung disease (group 3), CTEPH and other PA obstructions (group 4), and PH with unclear and/or multifactorial mechanisms (group 5).
Forty-three years old female was admitted with complaint of palpitation and shortness of breath. She has had symptoms for a year and the New York Heart Association functional capacity was IV. There was nothing to record on her background. On physical examination, increased second heart sound, 3/6 systolic ejection murmur on right parasternal area were recorded. Other system examination was normal. The rhythm was sinus and there was right axis and right bundle branch block on ECG. On chest X ray the pulmonary conus was prominent, the blood tests were in normal range except increased NT-proBNP levels (330.5 pg per mililiter). TTE revealed right heart chamber dilatation, secundum ASD and PH (systolic PAP:50 mmHg). The pulmonary:systemic flow ratio was 2.5. TEE confirmed the secundum ASD (3.2 cm) (Figure 1). Table 1. is showing the right heart catheterization results. We decided not to close the ASD due to high PVR (>4.6 WU). Patient who has 434 meter six-minute walking distance (6MWD) and FC III, was started masitentan 10 mg once a day. Tadalafil 40 mg once a day was added sequentially due to inadequate treatment response with monotherapy. After oral double combination therapy, the 6MWD was increased to 500 meter, and FC was improved as II. RHC was repeated and it revealed PVR as 1 WU (Table 1). According to results of second RHC, ASD was planned to close with perforated patch by surgery because there was prominent right to left shunt (Qp/Qs:2.4) and treatable PVR threshold.
Congenital heart disease (CHD) especially which causes right to left shunt like ASD may manifest with PAH associated with CHD (APAH) in if they are not diagnosed and treated in timely manner. And if PAH is suspected after transthoracic echocardiography, RHC should be performed in experienced center. Atrial septal defect is not recommended to close if PVR value exceeds 4.6 WU. In this situation PAH specific therapy should be started and goal oriented therapy should be followed according to currents ESC guideline on diagnosis and management of PAH. If the low risk criteria could not be reached by monotherapy, sequential combination therapy should be rapidly started. The risk situation should be checked hemodynamically by RHC in follow-up and if the PVR value which has no contraindication to close could be reached, ASD should be closed according to ‘treat and repair strategy’ by heart team decision.