SOMETIMES, TWO CARDIAC PATHOLOGIES ARE BETTER THAN ONE

Introduction: Eisenmenger syndrome is a severe complication of adult congenital heart defects, particularly those involving communication between the heart chambers or large vessels, and typically develops between 20 and 30 years old if the defect remains untreated.

Methods: We present the case of a 47-year-old patient, a smoker, a chronic ethanol abuser, hypertensive, and non-compliant to therapy, arriving at the emergency room due to right anterior epistaxis and symptomatic elevated blood pressure values (220/120 mm Hg). The patient stated that he had noticed the presence of low exercise tolerance over time, but he was not evaluated by a cardiologist. The emergency FoCUS echocardiographic examination performed in the emergency room apparently showed a hypertensive heart.

Results: After the correction of his high blood pressure, the patient was hospitalized. On clinical examination, the detection of a loud systolic murmur in all auscultation points and his ECG result (a right hyperdeviated electrical axis and the criteria for biventricular hypertrophy) led to the standard transthoracic echocardiographic re-evaluation. It showed severe left and right ventricle hypertrophy, a very small right ventricle cavity, and a membranous ventricular septal defect (with an 8 mm left-to-right shunt). No significant anomalies of the aortic and pulmonary valves were detected. The results lead to several questions. Is the right ventricle hypertrophy secondary ? Why is the interventricular shunt still left–right at his age and in spite of arterial hypertension and the large ventricular septal defect? Why has he not developed Eisenmenger syndrome? Why is there severe biventricular hypertrophy? Is there associated hypertrophic cardiomyopathy?

Conclusions: The imaging data provided by standardized transthoracic echocardiography require the addition of other imaging and paraclinical methods for an integrated, multimodality imaging diagnosis. In echocardiography, the detection of one congenital heart disease obliges us to search for a second. Sometimes, two cardiac pathologies are better than one.