Medical history and current illness
61-year-old male, ex-smoker of 10 cigarettes a day for 5 years, hypertensive and dyslipidaemic under pharmacological treatment. He denied a family history of heart disease. A sister of his had only one kidney since birth and a niece had been diagnosed with polysplenia since childhood. Cardiologically, he had been diagnosed 5 years ago with dilated cardiomyopathy in the context of a routine medical check-up, being completely asymptomatic, NYHA functional class I. He had a history of dilated cardiomyopathy. It was a dilated cardiomyopathy with moderate left ventricular dysfunction, with normal coronary arteries and associated mild aortic stenosis and moderate MI due to P2 prolapse.
He was asymptomatic and under medical treatment until four years after diagnosis, when, following the onset of moderate exertional dyspnoea, an echocardiogram was performed which revealed severe MR. He underwent surgery with Carpentier mitral annuloplasty.
After 3 months, he was readmitted for heart failure with moderate MR that became severe on stress echocardiography. At that time, reintervention was discouraged given the history of previous surgery and the presence of severe ventricular dysfunction. After three months he fell into AF, which could not be cardioverted chemically or electrically, so he was anticoagulated and managed with rate control. He was referred for evaluation of cardiac transplantation.

Complementary tests
- ECG: AF and a ventricular response at 80 bpm with a narrow QRS were observed.
- Chest X-ray: increased CTI with no evidence of pulmonary congestion. The blood test showed an NTproBNP of 14000, with no other findings of interest. Serology was positive for Toxoplasma IgG and CMV IgG and negative for hepatitis virus and HIV. He had a positive anti-lymphocyte antibody panel of 20%.
- Echocardiogram: a dilated LV with an EF of 25%, moderate-severe MR and severe low-gradient aortic stenosis that had not been described in previous echocardiograms (AVA 0.5 cm2; mean gradient 20) with contractile reserve was described.
- Ergometry with gas consumption: estimated VO2 PEAK of 8.5 ml/kg/min (20% of the maximum theoretical value). Weber-Janicki functional class: D. In the 6-minute test he performed 320 metres without stopping or desaturation.
- Right catheterisation: showed a PCP of 32, a PAP 68/32/44, and a cardiac index of 2.6 l/min/m2.
- Screening with thoracoabdominal CT (with a view to probable cardiac transplantation): showed polysplenia, hepatic and splenic inversion and anomalies in venous drainage (left superior vena cava draining into the coronary sinus; absence of vena cava above the renal veins, draining the entire infrahepatic venous system directly into the right superior vena cava via the azygos system.

Clinical course
Without complications during admission, the case was discussed in a medical-surgical session. Aortic and mitral prosthesis surgery was considered an excessive risk and the patient was accepted as a candidate for heart transplant on the elective list.

Diagnosis
- Chronic heart failure in advanced NYHA functional class III, with evidence of poor prognosis
- Dilated cardiomyopathy of probable valvular origin
- Carpentier annuloplasty one year ago
- Recurrence of severe MI and current severe aortic stenosis
- Severe systolic ventricular dysfunction
- Pending decision to be placed on elective cardiac transplant waiting list Polysplenia, hepatic inversion, situs sólitus
- Left superior vena cava and venous drainage anomalies
- Previous
