Congenital unilateral absence of pulmonary artery (UAPA) is a rare malformation– 0,39% of congenital heart diseases, may involve right or left branch. Absent left pulmonary artery is more often associated with congenital heart diseases while absent right pulmonary artery usually is present as an isolated form. Early diagnosis of UAPA, especially of the isolated form is very important because of later consequences.
Aim: Having our own material as well as publicated date we present symptoms, clinical date and diagnostic methods useful in making diagnosis of UAPA as well as therapeutic procedures.
Material and methods: We analyzed the group or 6 patients (3 children and 3 adults). X-ray, ECG, ECO were done in all cases, in some of them we performed angioTK, MRI and hemodynamic investigation.
Results: UAPA was recognized in all cases, isolated absence of RPA in one case, absence of LPA was in 5 cases and in 4 of them was associated with congenital heart diseases. There was diminished pulmonary marking on X-ray in all patients as well as ECO examination revealed possibility of UAPA. In 2 cases the diagnosis was established during catheterization and in other cases angioCT or MRI was done. In one patient three interventional procedures were performed.
Conclusions:
1.The clinical date of UAPA are not specific. In cases with associated congenital diseases the most characteristic are symptoms caused by this malformation.
2.The early diagnosis is done on the basic of X-ray and ECO. MRI, angioCT and hemodynamic investigation allow to establish final diagnosis.