Rare causes of pulmonary hypertension: spectrum of radiological findings and review of the literature.

Following a brief introduction covering the clinical signs and symptoms of pulmonary hypertension (PH), its most recent classification into six groups, and the computed tomography (CT) features common to all forms of PH, this paper illustrates the typical patterns that can be found on chest radiography and CT in rare causes of PH. We present and compare with the existing literature our personal series of cases of rare forms of PH, found in the following diseases: veno-occlusive disease, pulmonary capillary haemangiomatosis, non-thrombotic pulmonary embolism (tumour embolism and carcinomatous lymphangitis, talcosis, hydatid disease), pulmonary artery sarcoma, neurofibromatosis, sarcoidosis, and Langerhans cell histiocytosis. Rare forms of PH show low incidence and prevalence, and are, therefore, poorly recognised. Their diagnosis is a challenge for clinicians, pathologists, and radiologists, and any additional knowledge about the CT findings may help the diagnosis in the case of patients affected by PH of unknown origin.

[Rescue thrombolytic therapy for the treatment of ST-elevation myocardial infarction after unsuccessful primary percutaneous coronary intervention in a patient with coronary artery aneurysm]. / Infarto miocardico acuto trattato con trombolisi di salvataggio dopo inefficace angioplastica coronarica primaria in paziente con aneurismi coronarici.

The recommended treatment for ST-segment elevation myocardial infarction (STEMI) is primary percutaneous coronary intervention (pPCI). However, in a non-negligible proportion of patients, pPCI is ineffective and the cardiologist must face the decision of how to achieve optimal myocardial reperfusion. Although the possibility of a rescue fibrinolytic strategy has not been evaluated yet in this clinical setting, it is a viable alternative to emergency cardiac surgery. We here report the case of a 60-year-old STEMI patient presenting with a coronary anatomy unsuitable for percutaneous mechanical revascularization, characterized by marked dilation and tortuosity of the proximal and middle epicardial segments. After pPCI failure, the administration of recombinant tissue-type plasminogen activator allowed us to obtain reperfusion as shown by clinical outcome, ST-segment resolution and subsequent angiographic study. No indication was given to further percutaneous or surgical revascularization. The long-term pharmacological management of these patients represents a challenge for the clinician, also considering the available data on the use of new antiplatelet and anticoagulant molecules and their possible associations.

[Pulmonary arterial hypertension. Part II: Medical and surgical treatment]. / L'ipertensione arteriosa polmonare. Parte II: Terapia medica e chirurgica.

Treatment of pulmonary arterial hypertension (group 1 of clinical classification) has been recently characterized by important progresses, particularly in pharmacological therapy. Only until few years ago, patients with pulmonary arterial hypertension were treated with non-specific drugs, such as diuretics and digoxin for right heart failure and calcium-channel blockers in the minority of cases, responders to the acute vasoreactivity test. In addition, use of oral anticoagulant treatment was supported by uncontrolled studies. In the last 15 years (in particular in the last 8 years) different randomized controlled trials assessing the functional, clinical and hemodynamic efficacy of three classes of targeted drugs (prostanoids, endothelin receptor antagonists and phosphodiesterase type 5 inhibitors) with pulmonary vascular dilating and antiproliferative effects have been performed. This information has allowed the proposal of an evidence-based treatment algorithm. Treatment starts with general measures (physical activity, fertility control, respiratory tract infection, etc.) and supportive therapy (anticoagulant therapy, diuretics, oxygen, digoxin). Patients who respond to the acute vasoreactivity test (10% of idiopathic form) are treated with high doses of calcium-channel blockers, non-responders with targeted therapies either on monotherapy or combination. Usually an oral active drug is initiated and a second compound of a different class is combined in case of non-satisfactory response to the first treatment. Combination therapy should be performed only in specialized centers with large experience on use of targeted therapies and their relevant side effects. In case of failure of medical therapy, possible options are balloon atrial septostomy and/or listing for lung or heart-lung transplantation. As available treatments do not constitute a cure for pulmonary arterial hypertension, further progresses are expected in the near future.

[Pulmonary arterial hypertension. Part I: pathobiologic, pathophysiologic, clinical and diagnostic aspects]. / L'ipertensione arteriosa polmonare. Parte I: patobiologia, fisiopatologia, aspetti clinici e diagnostici.

Pulmonary hypertension is a pathophysiologic condition characterized by the increase of mean pulmonary arterial pressure > or =25 mmHg. A concomitant increase of pulmonary wedge pressure >15 mmHg may be present (post-capillary pulmonary hypertension) or not (precapillary pulmonary hypertension). The increase of pulmonary arterial pressure and of pulmonary vascular resistance and consequent elevation of the right ventricular afterload lead to right ventricular failure after variable periods of time. Pulmonary hypertension is present in multiple clinical conditions which have been classified in five groups. Pulmonary arterial hypertension (group 1) includes the familial and the idiopathic form and the forms associated with anorexigen drug use, connective tissue diseases, congenital heart diseases, HIV infection and portal hypertension. Group 2 includes all left heart diseases characterized by the increase of left atrial pressure and pulmonary wedge pressure (post-capillary pulmonary hypertension). Group 3 includes parenchymal lung diseases (chronic obstructive lung disease, lung fibrosis, ecc). Chronic thromboembolic pulmonary hypertension (group 4) is characterized by the obstruction of elastic pulmonary arteries at different levels by organized thromboembolism. Group 5 includes heterogeneous conditions such as sarcoidosis and histiocytosis X. These clinical groups are characterized by different pathobiologic and pathophysiologic mechanisms and therapeutic strategies. The exact pathobiologic mechanisms leading to pulmonary arterial hypertension (group 1) are unknown. Genetic factors (inheritable forms), predisposing factors (female gender) and exogenous factors (drugs, antibodies, viruses, congenital heart disease, etc). Endothelial dysfunction of lung microcirculation is invariably present and is characterized by the reduction of vasodilator and antiproliferative substances (prostacyclin, nitric oxide) and by the increase of vasoconstrictor and mitogenic factors (endothelin, thromboxane A2). Current approved therapies are targeted to the correction of this imbalance, which leads to the progressive increase of pulmonary vascular resistance. Different therapeutic strategies that are effective in diverse groups require an appropriate diagnostic algorithm in order to identify the precise group and specific conditions within the group. Evaluation of vasoreactivity and assessment of the severity of functional and hemodynamic changes are also required in pulmonary arterial hypertension for an appropriate therapeutic decision-making and estimate of results.