Pulmonary stenosis (PS) is mainly a congenital defect that accounts for 7–12% of congenital heart diseases (CHD). It can be isolated or, more frequently, associated with other congenital defects (25–30%) involving anomalies of the pulmonary vascular tree. For the diagnosis of PS an integrated approach with echocardiography, cardiac computed tomography and cardiac magnetic resonance (CMR) is of paramount importance for the planning of the interventional treatment. In recent years, transcatheter approaches for the treatment of PS have increased however, meaning surgery is a possible option for complicated cases with anatomy not suitable for percutaneous treatment. The present review aims to summarize current knowledge regarding diagnosis and treatment of PS.
Cardiac ventricular outpouchings and invaginations are rare structural abnormalities and usually incidental findings during cardiac imaging. A definitive diagnosis is possible through the use of multimodality imaging. A systematic review of the literature was carried out in November 2022 to identify studies regarding ventricular outpouchings and invaginations. The main aim of the review is to summarize knowledge regarding epidemiology, etiology, diagnosis and prognosis of patients with ventricular outpouchings (aneurisms or diverticula) and invaginations (crypts and recesses). Overall, 26 studies published between 2000 and 2020 were included in the review. Diverticula and congenital aneurysms incidence ranges between 0.6 and 4.1%. Myocardial recesses and crypts range between 9% in the general population and up to 25% in patients with hypertrophic cardiomyopathy. The combined use of echocardiography, cardiac computed tomography (CCT) and cardiac magnetic resonance (CMR) is useful to establish tissue contractility, fibrosis, extension and relationship with adjacent structures for differential diagnosis of both invaginations and outpouchings. In conclusion, both outpouchings and invaginations are rare entities: a definitive diagnosis may be aided by the use of combining multiple imaging techniques, and the treatment depends both on the lesion-specific risk of complications and on the potential association of some lesions with cardiomyopathy.
We reported a case of 46–year–old women without cardiovascular risk factors, with a diagnosis of relapsed metastatic melanoma undergoing treatment with immune check–point inhibitors (ICI), ipilimumab and nivolumab. A transthoracic echocardiogram was performed before starting the treatment that showing normal indices of biventricular function and a normal value of global longitudinal strain (GLS). After the third immunotherapy cycle, she presented to the hospital for diplopia and blurred vision; in the suspicion of ICI–related toxicity, muscle–specific enzymes and troponin I high sensitivity (TnI hs) were assayed and were found elevated (TnI hs 712 ng/L). Brain natriuretic peptide (BNP), electrocardiogram (ECG) and echocardiogram were within the limits. Given the positivity of cardiac markers in an asymptomatic patient, it was concluded for grade I ICI–related toxicity. Immunotherapy was discontinued and the patient was admitted to the cardiology intensive care unit. She underwent telemetry monitoring and a cardiac magnetic resonance (CMR) were performed showing a thin stria of sub–epicardial oedema along anterior wall without late gadolinium enhancement or altered kinetics. During hospitalization, patient was treated with methylprednisolone 1g i.v. for the first days with benefit and she was discharged with oral prednisolone, reducing the dose by 10 mg/week under clinical, ECG and TnI surveillance. I.v. immunoglobulins were started due to myopathy involvement. The tapering of corticosteroids after 15 days caused a flare–up of troponin levels, which resolved after increasing the steroid dosage. After a thorough discussion between experts, it was decided to resume ICI treatment. Myocarditis is a severe complication of ICI that occurs in 0.27–1.14% of patients and develops early. Surveillance should be done by biomarker assay, as major cardiac events occur in up to 40% of patients with normal ejection fraction. The clinical diagnosis includes the cTn elevation with 1 major criterion (CMR positive for Lake Louis criteria) or 2 minor criteria. The treatment strategy consists of interruption of ICI therapy, immunosuppressive agents and cardiac monitoring. Current guidelines recommend permanently ending ICI therapy when patients develop grade 3 or 4 toxicities; conversely, it may be considered again in case of grade 1 (asymptomatic biomarker elevation) or 2 (biomarker elevation and mild symptoms) toxicity.
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