All roads lead to Rome

Except for the submarine route leading to Stockholms Skärgård. And so was the story of a kind 80-yo lady with unique attributes presenting to her family physician for a routine check-up. She had a past medical history of asthma treated sporadically with bronchodilators. Upon cardiac auscultation a pan-systolic and diastolic murmur was audible along the left sternal border. Severe pitting edema of the lower extremities was present and in spite of a flat abdomen, the liver edge could be palpated well beyond its normal location. Lungs were clean as a whistle. She was referred for an ultrasound, and this was where I first had the joy of making her acquaintance.

As I walked into the examination room I saw the patient with her back turned towards me. I greeted her but received no response. When I placed my hand on her shoulder asking if she could lay down on the bed, she jumped, produced an ear-to-ear smile and turned red as a rocket. I was stunned at the behavioral constellation. She had quite the severe hearing problem and throughout our dialogue kept flushing red whenever I said something she didn’t catch.

Parasternal long-axis seems all right. How about the four-chamber view? Let’s have a closer look at the tricuspid valve.

Four-chamber view and parasternal long-axis tricuspid valve view. PLAX TV-view can be put into focus by directing the ultrasound waves caudally and towards the right hip. The tricuspid valve appears frozen in time. With an echolucent glaze it produces a severe tricuspid regurgitation. Remember the standard colordoppler setting BART: blue away, red towards.

Parasternal long axis with alternate pulmonary valve view appreciated by leaning the probe in a cranial- and left axillary direction. Same thing here: pulmonary valve is thickened and retracted. The result is a severe pulmonary regurgitation and mild pulmonary stenosis.

The subcostal view through the liver can clearly identify how parts of right ventricular endocardium is fibrotic. The right heart works like an open conduit pumping blood both ways while the left heart is largely unaffected. These are rare findings but almost pathognomonic for carcinoid heart disease. Carcinoid tumors, known as neuroendocrine tumors (NET), are found in the small intestine distal ileum and appendix nine out of 10 times, causing carcinoid heart disease in about half of them. The tumor secretes catecholamines and serotonin. Interestingly these biogenic amines are metabolized and largely inactivated in the liver, lung and brain. Hence, an effected right heart would signify metastasis to the liver. When the left heart is effected one must rule out primary tumor of to the lung, metastasis to the lung, right to left heart shunt or very high tumor activity. The latter was induced iatrogenically back in the 70ies with the drug combo fen-phen (Fenfluramine-Phentermine), used as an appetite suppressants for the treatment of obesity. Our lady had the age to match but I’m not sure how vigorously these drugs were prescribed in Sweden. In addition the left-sided valves looked good. She was referred for a computed tomography.

There seems to be an unusual flame-like contrast-load in the liver. A tumor-mass is present in the lower abdomen and can also be visualized by ultrasound.

Lab-tests for NET were ordered including chromogranin, tachykinin, urinary 5-HIAA, showing very high levels of 5-HIAA. This was a clear-cut case, or so it seemed. When I presented the news of right heart failure and suspected metastatic cancer to my patient she wasn’t alarmed. With age and resulting enlightenment already taken into consideration, I still expected at least a flinch. I kept huffing and puffing and nothing happened. With a jolly old smile on her face, intermittently flashing red, she strolled on happy throughout our conversation. Could it be that she’d become habituated to a constant surge of serotonin? This evokes the question of how her quality of life would be affected if serotonin levels suddenly dropped.

Even though malignant, neuroendocrine tumors tend to have a slow progression. When carcinoid heart disease is present, heart failure generally tends to be the cause of death and not the metastasized cancer. Mainstay of treatment is somatostatin analogue therapy and tumor surgery. Some cases are be eligible for valve replacement in spite of liver metastasis. In the end our patient decided against the valve surgery, but for the removal of the primary tumor. PET-scan and magnetic resonance imaging was performed priorly.

During exploratory laparotomy, the PET-scan finding was confirmed. The tumor was connected to the right ovary. Postoperatively somatostatin analogues were withdrawn and urine-5HIAA stayed normal signifying absent tumor activity! The carcinoid tumor was a primary gonadal tumor and not intestinal! The blood was draining to the right ovarian vein, connecting to the inferior vena cava, bypassing the liver and producing right-sided carcinoid heart disease. Such a case has only been described anecdotally in the literature! Let’s look at the blood flow through the liver.

In the subcostal view we can see the liver, inferior vena cava and severely dilated hepatic veins. The venous blood, identified by the colordoppler, is pushed back and forth through the IVC and hepatic veins due to the failing tricuspid valve. Very large amounts of blood are regurgitated and produce severe liver congestion. This might help us understand how the CT-findings looked so magnificent, when in fact, there was no metastasis.

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