Cardiac MRI in acute heart failure complicated by scleroderma renal crisis from the viewpoint of cardiorenal syndrome

Description of Clinical Presentation: A 49-year-old man with systemic scleroderma was treated with Bosentan Hydrate for skin ulcers and the ulcers improved. He was subsequently referred to our hospital for hyperbaric oxygen therapy due to a reoccurrence of exacerbation of skin ulcers on both lower legs. On admission, laboratory tests showed worsening renal dysfunction with a Cre of 2.29 mg/dl and eGFR of 25.6ml/min/1.73.In addition, acute heart failure was suspected and the patient was referred to our department based on the findings of cardiac enlargement and congestion on chest X-ray, in addition to a highly elevated blood pressure. Transthoracic echocardiography suggested diffuse wall motion decline and pericardial effusion.The patient had scleroderma renal crisis and cardiac failure findings and was treated with antihypertensive therapy with calcium channel blockers and Renin-Angiotensine system (RAS) inhibitors, without diuretics, as there was no poor oxygenation. A cardiac MRI scan was performed to further understand the cardiac morbidity.

Diagnostic Techniques and Their Most Important Findings: The patient was taken non-contrast-enhanced due to renal dysfunction and showed a mildly decreased LVEF of 51% and mild or slightly enlarged left ventricular wall thickening. A circumferential pericardial effusion and bilateral pleural effusions with left predominance were noted.T1 mapping showed a global native of 1200 ms, an increase of more than 2 SD of the institutional standard, and T2 mapping showed a similar increase of 66 ms, suggesting interstitial disorders and edema. After three weeks of adjustment of antihypertensive medication, blood pressure control improved, and renal dysfunction mildly enhanced. The pleural effusion findings on chest X-ray also improved without diuretics, and a repeat cardiac MRI three weeks later showed mild improvement of the above findings.Acute myocarditis was ruled out due to the absence of ECG changes and elevated inflammatory response on blood tests. There were also findings of haemolytic anaemia and thrombotic microangiopathy at the onset of scleroderma renal crease, suggesting that microvascular damage and spasm may have been caused not only in the kidney but also in the heart. If we interpret the present pathology in a monolithic way, the elevated blood pressure associated with scleroderma renal crisis led to an afterload mismatch, which was thought to be mainly due to acute myocardial edema.

Learning Points from this Case: The acute phase of scleroderma renal crisis has previously been reported to present with pericardial and pleural effusions. There have been no previous reports assessing myocardial properties in the acute phase by parametric mapping. This is a valuable report of subacute improvement only by adjusting cardioprotective antihypertensive drugs without diuretics, and the use of parametric mapping by cardiac MRI is important to understand the pathology.

Comparison of short-axis and LVOT images of Cine at baseline Pre and after 3 weeks.

Comparison of basal short-axis images of T1/T2 mapping at baseline Pre and after 3 weeks.

Comparison of baseline Pre and T1 polar map at 3 weeks.