ACD: An interesting case

of dyspnea4/9/2015

Rahul Ravilla

PGY3

Simulated Case presentation

55 year old woman presents with shortness of breath.

Past medical history

VSD repair at age of 4

Liver cirrhosis

DM type 2

Chronic back pain

Neuropathy

Past surgical history

Bilateral Knee surgery

Bilateral shoulder surgery

Hysterectomy

Bilateral oophorectomy

Review of systems

Abdominal pain- Right upper quadrant

Lower extremity pain

Orthopnea

Lower extremity swelling

Shortness of breath/ Dyspnea on exertion- NYHA Stage 4.

Physical examination

BP 110/64 | Pulse 80 | Temp(Src) 98.4 °F (36.8 °C) (Oral) | Resp 22 | Ht 5' 4" (1.6 m) | Wt 190 lb

(86 kg) | BMI 33 kg/m2 | SpO2 95% on RA

Gen: overweight white female appears stated age, slightly jaundice, AAOx3, NAD

HEENT: normocephalic, atraumatic, dry mucus membranes, no scleral icterus, PERRLA, EOMI

Neck: no LAD, huge venous pulsations in the right neck

Lungs: CTAB

Heart: RRR, S1, S2, Harsh systolic ejection murmur at RUSB, radiating to carotids, notable

carotid pulsations (pulsating 1-2 inches from sides of neck)

Abd: obese, non-tender, mildly distended, non-tense, NBS

Ext: Presacral edema and 4+ peripheral edema to thighs

Neuro: intact

Skin: no rashes or lesions

Laboratory work up

CBC- 5.0/8.0/90

BMP- 128/3.6/92/ 28/42/0.9 Ca- 9.1 Po4- 4.4, Mg- 1.6, Gluc-157

BNP- 200

Albumin- 3.3

T bil- 2.3, D bil- 0.6, AST-90, ALT- 20, Alk phos- 110, GGT- 80, LD- 150

INR- 1.28

Abg- 7.46/38/80 on room air

What would you do at this point?

PT gets worked up at OSH then sent to you.

Work up at OSH

Transthoracic echocardiogram

CT CHEST

Treated for CAP

Medications- Furosemide 40mg PO, Spironolactone, HCTZ, Propranolol,

Sertraline, Oxycodone.

Work up at UAMS

Transthoracic echo- Normal EF of 60%, No AS, No MR, Ventricular septum

flattening, Dilated RA and RV, normal tricuspid valve with leaflet separation,

Moderate to Severe TR.

Bubble study- There was a mild right-to-left shunt, in the baseline state( few

bubbles appear in the left heart at the 10th cardiac cycle). This is consistent

with an extracardiac shunt such as a pulmonary AVM.

ECG- next slide

CT Chest/Abdomen

What is the diagnosis?

What test will confirm?

Right heart catheterization

LEFT HEART HEMODYNAMICS (mmHg):

AO : 108/46/54

LV Systolic : 104

LVEDP : 24 (5-12mm)

RIGHT HEART HEMODYNAMICS (mmHg):

Right atrium - 38/14, Mean 21; O2 sat 58% (0-8mm)

Right ventricle - 46/15; Mean 19; O2 sat 55.1% (15-30mm/3-12mm)

Pulmonary artery - 44/26; Mean 36; O2 sat 52.6%

PCWP - 25/33; Mean 27; O2 sat 92.3% ( 3-15mm)

Fick Cardiac output - 4.18 L/min (4-6.5 L/min)

Cardiac index - 2.22 L/min/m2 (2.6-4.6)

How would you interpret that right heart

cath? What is causing the right sided

increased pressure?

Probably the best place to start when there are elevated pressures is the left

ventricle end diastolic pressure. LVEDP normal ranges are 5-12mmHg, and the

value of 24 in this case is markedly abnormal. Abnormally high LVEDP indicates

presence of left heart failure which is the cause of right heart and wedge increased

pressures. In this case, the severe tricuspic regurgitation is worsening the right

heart failure symptoms and causing the huge neck vein pulsations.

So what is the treatment?

How do you treat this patient with left heart

failure and tricuspid regurgitation?

Lots of loop diuretics. Patients like these may get 10-20 liters of diuresis

prior to significant improvement.

If left heart failure is stretching apart the RV and causing tricuspid regurg,

sometimes adequate diuresis can shrink the heart down and significantly

improve the regurgitation.

Tricuspid regurgitation Causes Functional TR- Valve anatomy is normal. Caused by dilation of RA and RV dilating the TR annulus ~ 70%

from causes of elevated pulmonary HTN.

Left-sided heart failure.

Mitral stenosis or regurgitation.

Primary pulmonary disease – cor pulmonale, pulmonary embolism, pulmonary hypertension of any cause.

Left to right shunt – atrial septal defect, ventricular septal defect, anomalous pulmonary venous return.

Eisenmenger syndrome.

Stenosis of the pulmonic valve or pulmonary artery.

Hyperthyroidism.

26% have normal pulmonary artery pressure

<2% have Atrial fibrillation

TR Causes: Intrinsic Valve defects

Direct valve injury

Chest trauma

Infective endocarditis

Ebstein's anomaly

Rheumatic fever

Carcinoid syndrome

IHD of RV damaging papillary musculature

Marantic endocarditis in SLE, RA

Drug induced like Fenfluramine, Phentermine

Indications for surgery For patients undergoing left-sided valve surgery:

For patients with severe tricuspid regurgitation (TR) who are undergoing left-sided valve surgery, tricuspid valve surgery is recommended,

For patients with mild, moderate, or greater functional TR who are undergoing left-sided valve surgery, concomitant tricuspid valve repair is suggested if there is either 1) tricuspid annular dilation (diameter on transthoracic echocardiogram of >40 mm or 21 mm/m2 indexed for body surface area or intraoperative diameter>70 mm) or 2) prior evidence of right heart failure.

Isolated tricuspid surgery –

Tricuspid valve surgery is suggested (weak recommendation) for patients with severe primary TR with symptoms unresponsive to medical therapy, preferably before the onset of significant right ventricular dysfunction, Patients with severe congestive hepatopathy may benefit from surgery to prevent cirrhosis.

The 2012 ESC valvular guidelines include a strong recommendation for tricuspid valve surgery in patients with symptomatic severe isolated primary TR without severe right ventricular dysfunction.

The role of tricuspid valve surgery in patients with severe TR with no or minimal symptoms is uncertain. Surgical treatment of moderate to severe TR may be helpful in patients undergoing pericardiectomy for constrictive pericarditis, although supporting data are limited.

References

Long-term prognosis of isolated significant tricuspid regurgitation. Lee JW,

Song JM, Park JP, Lee JW, Kang DH, Song JK. Circ J. 2010;74(2):375.

2014 AHA/ACC guideline for the management of patients with valvular heart

disease: a report of the American College of Cardiology/American Heart

Association Task Force on Practice Guidelines. Nishimura RA et al J Am Coll

Cardiol. 2014;63(22):e57