GDS, 38y, male, a schistosomiasis carrier that had been followed at PROCAPE-UPE outpatient clinic since 2003, when he presented breathlessness for great efforts and a syncope episode eight years before first care.
At this time, the physical examination showed a loud P2 and moderate regurgitation murmur in the left lower sternal border (LLSB). On EKG, right atrial and ventricular overload, with strain pattern. On 4th march 2005 he was submitted on a hemodynamic study: mPAP=62mmHg, SPAP= 90mmHg; OPCP=8mmHg; mRA= 10 mmHg; PVR=1437dyn/seg/cm-5 and CI=2,21l/min/m².
He was started on sildenafila 40mg three times/daily; reported feeling better. In 2007 he was elegible to EARLY study, adding bosentan to his therapy and, since then, remained in FC I.
On february 21th 2017 he was attended in an emergency room complaining of excruciating thoracic pain started three days before, that worsens in the last 12 hours.
The physical examination showed a respiratory rate of 24ipm, blood pressure = 110 x 70mmHg. A regular heart rhythm with a intense systolic murmur in LLBS and a diastolic murmur in the ULBS.
The lungs were clear and abdomen examination was normal.
By the possibility of acute pulmonary embolism it was requested a thorax angioCT-scan that showed a large pulmonary trunk dissection.
A echocardiogram showed a large “flapping” of intima inside pulmonar trunk, 3 cm below the pulmonary valve ring.and a severe pulmonar regurgitation.
The patient was submitted to thoracic surgery and it was seen the large progression of the dissection line. The pulmonary trunk was resected, and interposted a tube of dacron
The patient arrived to the ICU initially with stable haemodynamics conditions, but he died three days after by kidney dysfunction and cardiogenic shock.
Indeed, the dissection of pulmonary trunk is a very rare and highly lethal complication of pulmonary hypertension and, to date, there are only 26 reported cases in alive patients, justifying this presentation.