Persisting pulmonary arterial hypertension (PAH) after shunt closure is associated with poor prognosis. Patients with a large ventricular septal defect (VSD) may develop preoperative pulmonary vascular disease that can persist and progress despite shunt closure. A 28-year-old woman referred herself to our clinic to seek help to improve her exercise program. At the age of 12 months, a large perimembranous VSD had been closed. The preoperative right heart catheter(RHC) had showed a 36% left-to-right shunt and mean pulmonary artery pressure (mPAP) of 50 mmHg. Repeated transthoracic echocardiographies (TTE) in the following years were reported as normal. In the last three years, the patient reported a reduction in exercise tolerance with progressive breathlessness on exertion despite regular training, as well as central cyanosis and vomiting during exertion. TTE revealed severe dilatation of the hypertrophied right ventricle (RV) with severely reduced systolic function, as well as an elevated systolic PAP of 60 mmHg. Review of the TTEs from the last ten years performed in other institutions revealed that RV dilatation and markedly reduced RV function had been present for years; however, the transtricuspid gradient seemed to be normal (although the signals of the very mild tricuspid regurgitation were hard to measure). Pulmonary function tests revealed normal static and dynamic lung volumina but reduced diffusion capacity (65% of predicted). Peak oxygen uptake was severely reduced (16.1 ml/kg/min;50% of predicted) and minute ventilation/carbon dioxide production slope of 64 massively elevated. A peripheral oxygen desaturation from 96% to 74% was observed. RHC showed severe pre-capillary PH with mPAP of 79 mmHg and pulmonary vascular resistance of 21 Wood units. Lung scintigraphy showed no evidence of chronic pulmonary thromboembolism. Treatment with macitentan and riociguat was started. Four months later the patient reported significant symptomatic improvement in her daily activities, although the first follow up TTE did not reveal any relevant changes. However, B-type natriuretic peptide decreased from initially 114 ng/l to 64 ng/l. Our case shows that relevant PAH may develop and become clinically manifest decades after shunt repair supporting the hypothesis of slow progression of PAH. There is limited experience and long-term date available for this rare clinical problem. In the case of lack of improvement, heart and lung transplantation have to be discussed
A severe PAH revealing a veno-occlusive disease about a case.
Veno-occlusive disease (VOD) is a rare form of pulmonary hypertension characterized by predominant venous involvement associated with capillary proliferation. The last decade has been marked by the discovery of biallelic mutations responsible for heritable forms. Its evolution is shady and lung transplantation remains the reference treatment.
We report the case of a 25-year-old patient, without comorbidities, without toxic habits,
no concept of consanguinity and no family history who experienced NYHA Stage IV dyspnea. The clinical examination found a cyanotic patient with pronounced digital clubbing and crackling rales at the two pulmonary bases. O2 saturation with ambient air was 75%, therewere no signs of right heartfailure. The radiological assessment revealed the presence of an interstitial syndrome associated with bilateral hilar adenopathies and dilation of the trunk of the pulmonary artery. The echocardiography found HTP signs with good LV function. Right cardiaccatheterization confirmed the presence of severe precapillary PAH. Blood gasometry showed the presence of severe hypoxemia. Symptomatic treatment was instituted but the evolution was fatal in a few weeks.
An isolated case of MVO within a family may be the first case of an heritable form of the disease. The prevalence is unknown while its incidence is estimated at 0.5 cases / million inhabitants. Risk factors have been identified as chemotherapy, exposure to organic solvents and smoking. Diagnosis is based on chest CT, the DLCO, arterial blood gases and the identification of biallelic mutations of the EIF2AK4 gene for heritable forms. The prognosis is very restricted and the effectiveness of specific PAH treatments is limited.
MVO is a rare form of pulmonary hypertension. Its differential diagnosis with idiopathic PAH isdifficult because of their clinical and hemodynamic similarity.The use of specific treatments is controversial because of the risk of pulmonary edema that they can cause. The only cure is lung transplantation.
Acute pulmonary artery dissection in pulmonary arterial hypertension
A 65-year old female patient with known pulmonary arterial hypertension (PAH) presented with respiratory symptoms and an acute myocardial infarct was diagnosed. Because of respiratory and hemodynamic decompensation she was sent to the university hospital of Zurich and a computed tomography revealed an acute dissection of the pulmonary artery starting from the pulmonary valve and extending to the end of the right pulmonary artery. The main pulmonary artery was severely dilated to 8 cm. The patient was operated with a resection of the main and right pulmonary artery and with placement of a graft. Secondary to the dissection the main stem of the coronary arteries was compressed, resulting in a myocardial infarct with reduced heart function and cardiogenic shock. The patient developed multiple organ failure, sepsis and diffuse lung bleeding and died after 12 days.
The patient had been diagnosed with PAH in 1993 at the age of 41 years with an echocardiography. The right heart catheter was performed 2006 and showed a PAH fulfilling the vasoreactivity criteria (mean pulmonary arterial pressure (mPAP) 55mmHg, wedge pressure 6 mmHg, cardiac index (CI) 2.9 l/min/m2, pulmonary vascular resistance 12 WU, after iloprost inhalation mPAP 35mmHg, CI 4.7 l/min/m2). The echocardiography showed a severe dilatation of the main and right pulmonary artery since many years (main pulmonary artery dilated to 6.5 cm in 6/2017). She was treated with calcium channel blockers since 2006. 2012 bosentan was added to the therapy. She was in a good and stable condition (NYHA II) until the acute event.
Pulmonary artery dissection has been described in patients with long-standing PAH and is a life-threatening condition. The possible risk factors are preexisting dilatation, in-situ thrombosis or worsening of the PAH. Urgent surgical repair or heart-lung transplantation is required to prevent lethal bleeding but the majority of the cases ended fatally.
Case Report: Pulmonary Arterial Hypertension due to Congenital Heart Disease – Think of it in adults!
We present a 42-year old woman with severe pulmonary arterial hypertension (PAH) associated with a congenital heart disease.
A first consultation with a cardiologist took place at the age of 41 after experiencing tachycardia at rest and during exercise for several years. The echocardiography showed significant right ventricular impairment and a systolic right ventricular pressure of about 60 mmHg. The diagnosis of precapillary pulmonary arterial hypertension was confirmed in right heart catheterization. The cardiac index and the mixed venous oxygen saturation were high, as well as the shunt fraction in the hyperoxia testing, rising suspicion of a cardiac shunt. A CT-scan as well as a transesophageal echocardiography revealed a superior sinus venosus defect and partial anomalous pulmonary venous connection. A surgical repair (Warden Operation) was performed but mild PAH persisted which was treated with Sildenafil.
Lunch in the industrial exhibition
Salle des Abeilles
Satellite Symposium organized by Orpha Swiss
Salle des Abeilles
Form of presentation:
Vienna Long-term Experience with s.c. Treprostinil – Tips and Tricks