Focus on the snake & miss the scorpion

47-year-old woman seeking due to acute / subacute onset dyspnea.
Past medical history significant for rectal cancer with metastasis to liver, lungs and brain. Previously hospitalized due to severe left sided pleural fluid.
Current vitalsigns: Sat 91% on 2L oxygen, p.90, BP 115/70. The journal shows a habitual BP of 140/70. Clinical examination reveals absent leftsided breath sounds and dull percussion.

Spine-sign, well-defined diaphragm and the absence of curtain-effect indicates a large amount of pleural fluid. A fluid gap is also seen apically. But was this the entire truth? One would expect a gradual onset dyspnea in this setting.

The right ventricle, located adjacent to the liver, seems overloaded basally. Inferior vena cava (IVC) seems plethoric (dilated with poor respiratory variation) signifying increased central venous pressure (CVP). What happens to the right ventricle if preload / returnvolume is increased? Ask the patient to take a deep breath. This generally also improves the subcostal image quality. Alternatively, try passive leg raise.

The basal part of the right ventricle is considerably dilated while the apex is hyperdynamic. Known as McConnell’s sign, it may indicate massive or submassive pulmonary embolism. But the question is if large amounts of unilateralt pleural fluid can induce respiratory variations in right ventricular load. A quick review of the literature comes back empty.
A left-sided pleural chest tube was set up and 1000cc of clear-red liquid drain after which the drainage was stopped. This was followed up by 500cc / 4hrs to avoid complications such as reexpansion-related pulmonary edema. The patient experienced immediate symptomatic relief and was admitted for further treatment. Sat 94%, p.80 and BP 120/70.
After a total of 2.5L fluid was drained, the patient noted a swelling of the lower extremities. Ultrasound showed bilateral DVT in common femoral veins. Pending thoracic CT according to LE protocol, a new cardiac echo was performed.

McConnell’s sign is accentuated by inspiration.
Central right-sided pulmonary embolism. In view of the brain metastasis, a reduced dose low molecular weight heparin was instituted BID.

One may ask if the CT is really necessary in this case. Bilateral DVT was already diagnosed and thus the indication for LMWH present. However, it was still deemed reasonable to perform the CT since the diagnosis was not quite clear and it may affect duration of treatment with anticoagulation.

The Whistleblower

It was a sunny July afternoon and time to wrap up for the day. A quick glance at the patient list however, and before you knew it I was taking a history.
Intermittent lower leg edema the last couple of months in a 40-or so woman. Physically active every day. No other complaints. No past medical history and no medication. A pinch of short stature and a pinch of overweight. Vital signs were ok. Lower extremities slender. All in all currently asymptomatic and seemingly well. This was a clear-cut case of thoracic aortic aneurysm involving the roots of the coronary arteries. Now all I had to do was convince the surgeon to perform a composite graft implantation.
A quick physical revealed a diastolic decrescendo murmur 2/6 at IC2-3 left parasternal. No rumbling, anterior mitral leaflet Austin-Flynt murmur over the apex, but it was good enough to justify a bedside echo. Lo and behold, a 6cm wide ascending thoracic aortic aneurysm with a mild aortic insufficiency.

But let’s drop the cool. Was this simply an ad-hoc finding? What was I looking for with the ultrasound? To make things clear, a diastolic murmur always warrants further diagnostics. But was the absent lower extremity edema related to the TAA? Perhaps it was, perhaps it wasn’t, perhaps it was hormones. Her neck was a bit short. Webbing of the neck springs to mind, and as such Noonan and Turner syndromes. Just as in Marfan-, Ehler-Danlos- and Loeys-Dietz syndromes, diseases effecting connective tissue, there is a relation to pathology of the aorta. Did I think of this? No. Upon focused history regarding chest pain, burried beneath a thick layer of anxiety, in the trunk of an old caaar, much was revealed. Onset, location, duration, character, alleviating- & aggravating factors, accessory symptom and radiating pain. First onset one year ago, 1-4 times a week, increasing in frequency lately. Episodes described as sudden in onset, retro sternal, stabbing chest pain radiating to the right arm and back. Aggravate by lifting boxes at work or while straining upon defecation, 5-10 minutes of duration, alleviated by rest.  Accessory symptom of dispnea while lying supine, alleviated by lying in lateralt decubitus. Compression of adjacent organs? She admitted to rationalizing the pain because there was no radiation to the left arm.

Boy oh boy was I worried. A young, vital woman in her prime with an intermittently symptomatic TAA and open heart surgery around the corner. I’ll leave you suspended in mid-air to appreciate the emotional limbo. Goal systolic BP < 120 until then.