Riddler #5Riddler #5

20 y/o female in her 120 y/o female in her 1stst trimester, trimester, is referred to you for progressive is referred to you for progressive dyspnea. An echo reveals severe dyspnea. An echo reveals severe pulmonary hypertension. Right pulmonary hypertension. Right Ventricular systolic pressure is 90 Ventricular systolic pressure is 90 with a systemic pressure of 110 with a systemic pressure of 110 mm hg. No PE and no mm hg. No PE and no intracardiac shunt.intracardiac shunt.

Riddler #5Riddler #5

Which of the following is associated with Which of the following is associated with the lowest risk of Maternal Mortality?the lowest risk of Maternal Mortality?

A) Termination of pregnancyA) Termination of pregnancy B) Initiation of prostacyclin therapyB) Initiation of prostacyclin therapy C) Initiation of bosentan therapyC) Initiation of bosentan therapy D) Initiation of anticoagulation therapyD) Initiation of anticoagulation therapy E) Initiation of ACE inhibitor therapyE) Initiation of ACE inhibitor therapy Bonus: Two points:Bonus: Two points: What Super Bowl did What Super Bowl did

Doug Williams win and who received the Doug Williams win and who received the 80 yard bomb pass from him during the 80 yard bomb pass from him during the game?game?

Riddler #5 AnswerRiddler #5 Answer

Pt has a new diagnosis of Primary Pulmonary Pt has a new diagnosis of Primary Pulmonary HTNHTN

Whether primary or secondary, carries a 30%-Whether primary or secondary, carries a 30%-50% risk of maternal mortality50% risk of maternal mortality

Pt should be counseled against continuing the Pt should be counseled against continuing the pregnancypregnancy

Prostacyclin and Bosenten (pulmonary Prostacyclin and Bosenten (pulmonary vasodilators) should be used if pt refuses vasodilators) should be used if pt refuses terminationtermination

Anticoagulation should also be considered due Anticoagulation should also be considered due to in-situ thrombosisto in-situ thrombosis

ACEI and ARB’s are teratogenicACEI and ARB’s are teratogenic

Riddler #6Riddler #6

68 y/o male describes a 2 year 68 y/o male describes a 2 year history of insomnia that she history of insomnia that she attributes to an inability to get attributes to an inability to get comfortable in bed due to a “creepy comfortable in bed due to a “creepy crawly” sensation in her lower limbs. crawly” sensation in her lower limbs. Physical exam is normal.Physical exam is normal.– Absent during the dayAbsent during the day– Also bothersome when she sitsAlso bothersome when she sits– Walking relieves the sensationWalking relieves the sensation

QuestionQuestion

What is the most likely diagnosis? What is the most likely diagnosis? (1pt)(1pt)

Which of the following studies should Which of the following studies should be done next? (1pt)be done next? (1pt)– a) Serum ceruloplasmina) Serum ceruloplasmin– b) Serum ferritin levelb) Serum ferritin level– c) Urine porphyrin levelc) Urine porphyrin level– d) Nerve conduction studyd) Nerve conduction study– e) Magnetic resonance image of the braine) Magnetic resonance image of the brain