MRCP 2 Onexam.com
1. A 28-year-old woman was admitted to hospital during her 28
week of pregnancy complaining of dyspnoea. Her symptoms had become increasingly more debilitating over a period of eight weeks. On the day of admission she felt dyspnoeic at rest, and was unable to lie flat. There was no past history of note, other than an admission for repair of a dislocated lens two years previously. This was her first pregnancy. Urine dip-stick was negative for protein. Inpatient investigation culminated in cardiac catheterisation, the data from which are shown below: Anatomical site Superior vena cava Inferior vena cava Right atrium (mean) Right ventricle Pulmonary artery Pulmonary capillary wedge pressure Left ventricle Aorta Oxygen saturation (%) 76 72 74 75 74 98 97 Pressure (mmHg) End systolic/End diastolic 9 60/8 58/26 30 150/25 150/44
What is the most likely diagnosis? Ankylosing spondylitis Marfan's syndrome Relapsing polychondritis Syphilitic aortitis Systemic lupus erythematosus
The catheter data are characteristic of aortic incompetence: there is a wide pulse pressure in the aorta accompanied by a very high left ventricular end-diastolic pressure (LVEDP); a LVEDP greater than 20 mmHg is suggestive of irreversible LV dysfunction. All of the conditions listed are associated with aortic regurgitation. However, the clinical description best fits with Marfan's syndrome. Marfan's syndrome is a connective tissue disease inherited in an autosomal dominant manner. Clinical features include:
Arachnodactyly High-arched palate Lenticular dislocation Arm span greater than height Aortic incompetence. Page 1
T.T.O Trading (DENK PHARMA)
MRCP 2 Onexam.com
Cardiovascular complications of the disease limit the lifespan. Aortic incompetence is more likely to occur during pregnancy when there is a greater cardiovascular workload.
2. A 66-year-old woman was admitted to hospital following a collapse while...
Please join StudyMode to read the full document