A 77-Year-Old Woman With Carcinoid Syndrome and Severe Pulmonary Regurgitation | Patient Case Quiz
A 77-year-old Caucasian woman with past medical history relevant for carcinoid syndrome due to biopsy-proven grade 2 neuroendocrine tumor that had metastasized to the liver but of unknown primary origin, was referred to the cardiology clinic for further evaluation of severe lower extremity edema. Lower extremity edema was present when she was first diagnosed with carcinoid syndrome two years prior, but had resolved following treatment with octreotide. In the preceding six months, her lower extremity edema became progressively worse, despite increasing doses of diuretic.
- Other Medical History: Hypertension.
- Family History: Non-contributory.
- Social/Occupational History: The patient is a widow with two grown children. She lives alone with her two cats and is retired (former cook for a local hospital). She is a nonsmoker and social drinker (1-2 glasses of wine/week).
Bumetanide, potassium chloride, octreotide, spironolactone, simvastatin, benazepril, and aspirin.
The patient was seen and evaluated in the cardiology clinic with physical exam as below.
|Vital Signs||Temperature 37.2˚C; Pulse 83 bpm; Blood pressure 160/85 mmHg; Respirations 16 breaths/minute; Oxygen saturation 97% on room air; Weight 62 kg; Height 158 cm.|
|General appearance||Alert, healthy-appearing, and in no acute distress|
|Head and neck||Moderate jugular venous distention with a jugular venous pressure of ~10-12 cm of H20 and large C-V waves; normal carotid volumes with no delay.|
|Cardiac exam||No right or left ventricular heave; grade 2/6 diastolic decrescendo murmur best heard in the left second intercostal space; grade 3/6 holosystolic murmur best heard at the left lower sternal border that accentuates with inspiration; no S3 or S4.|
|Chest and lungs||Clear to auscultation bilaterally|
|Abdomen||Normal bowel sounds; no ascites; pulsatile liver edge, which is palpable below the subcostal margin.|
|Extremities||3+ pitting edema extending to the thighs bilaterally.|
Relevant Laboratory and Diagnostic Studies
Twelve-lead ECG demonstrated normal sinus rhythm with bifasicular block (right bundle branch block with left anterior fascicular hemiblock). A transthoracic echocardiogram was also performed, which was significant for severe tricuspid regurgitation with mild thickening of the valve leaflets, mild to moderate pulmonic regurgitation, mild right atrial enlargement, and moderate right ventricular enlargement with preserved function. These findings were considered to be consistent with carcinoid heart disease.
To better delineate the patient's carcinoid heart disease, a gadolinium-enhanced cardiac MR was obtained. This study revealed a mildly enlarged right ventricle (end diastolic volume index, 122 mL/m2) with preserved function (ejection fraction, 59%); moderate pulmonary valve regurgitation (regurgitant fraction, 24%) with mild stenosis (peak gradient, 16 mmHg); and moderate to severe tricuspid regurgitation by quantitative assessment. Diagnostic coronary angiography demonstrated a high-grade lesion (80% stenosis) in the proximal left anterior descending artery (LAD).
The patient was diagnosed with carcinoid heart disease involving the tricuspid and pulmonary valves. Carcinoid accretion resulted in severe tricuspid regurgitation with mixed pulmonary valve disease characterized by moderate to severe regurgitation with mild stenosis.
The patient was initially referred to surgery for valve replacement of both the tricuspid and pulmonic valves. However, given there was no more than moderate pulmonic regurgitation and concern that the patient would not tolerate a prolonged double valve surgery with bypass grafting of the LAD, it was decided to proceed with tricuspid valve replacement along with grafting of the LAD. Therefore, the patient underwent single-vessel bypass surgery with end-to-side grafting of the internal mammary artery onto the LAD, and placement of a 25-mm Carpentier-Edwards bioprosthesis (Edwards Lifesciences, CA) in the tricuspid position.
Due to continued symptoms of right-sided heart failure in the months following tricuspid valve surgery despite aggressive medical therapy, she underwent further investigations including echocardiography and cardiac MR which confirmed severe pulmonic valve regurgitation. Diagnostic cardiac catheterization confirmed severe PR with low cardiac output/index, (2.24/1.52 L/min via Fick) in the presence of normal tricuspid prosthetic function. Subsequent to this percutaneous placement of an Edwards SAPIEN XT transcatheter heart valve (Edwards Lifesciences, CA) in the pulmonic position was pursued. After obtaining informed consent from the patient for this "off-label" use of the transcatheter heart valve, the patient successfully underwent implantation of a 26-mm Edwards SAPIEN XT valve in the pulmonic position under general anesthesia and with transesophageal echocardiography for guidance, following balloon sizing of the pulmonic annulus with a 24mm sizing balloon without any complications. (Video 1 and 2) She was discharged to a rehabilitation facility two weeks later with much improved peripheral edema and stable renal function, after a brief period of continuous veno-venous hemodialysis due to anuria.
Transcatheter heart valve implantation is a viable alternative to surgical valve replacement for specific types of valvular heart disease, including native-valve aortic stenosis, right ventricular outflow tract/pulmonary valve conduit dysfunction, and bioprosthetic valve failure.
In this case, there was careful consideration about the next best step in management. Carcinoid accretion resulted in severe tricuspid regurgitation which was managed surgically with pulmonary valve disease characterized by progression to severe regurgitation and severe right heart failure. Ultimately, a hybrid surgical and percutaneous approach was chosen.
When considering a percutaneous approach to the treatment of valvular heart disease, there are several important factors that need to be evaluated.
Which of the following statements is not correct regarding TAVI?