Tamworth Cases • By Nick Jackson
Case 1 • 50 yo man transferred from a peripheral hospital with confusion, vomiting and blurred vision. • Na 118, K 3.7, HCO3- 24, Creat 80 • PHx: T2DM, Last HBa1c 8.9, Hypertension, hyperlipidaemia, GORD. • Meds: Felodipine, Metformin, Simvastatin, Pantoprazole, Irbesartan, Hydrochlorothiazide.
Examination • Euvolaemic. • Normal cardiorespiratory exam. • BP 110/60, P-65, 99% R/A • Initially was thought to have a bilateral 4th nerve palsy? • No other neurological findings.
Progress • Thiazide was stopped. • Sodium got a bit better, confusion persisted. • Urine Chemistry: Osmolarity 484, Na 6. • What next?
Investigations • Cortisol 44! (185-625) • What next? • TSH 0.22, T4 5.7, T3 4.2 • LH 1 (1.2-8.6), FSH 1.4 (1.3-19.3), Testosterone <0.4 (6.1-27.1) • Prolactin 338 (<280). • ACTH <1.1 (0-10).
Investigations • Next test?
Treatment? • Cortisone 25mg daily. • Neurosurgery. • ?Trans-sphenoidal adenectomy • Pituitary Apoplexy -Haemorrhage into an adenoma. Cavernous sinus cranial nerve palsies III, IV, V1, VI.
Case 2 • 72 yo man presented to a satellite hospital with chest pain. • Pain on and off for 4 days with worsening SoB and was transferred to Tamworth. • Maximum troponin 7.6 • ECG: SR, minor ST elevation in 3 and AvF with Q waves and LBBB.
PHx • pt had had intermittent cp and a dry cough for two weeks. • Phx: Htn, retinal haemorrhage. • O/E Pulse 90, BP 130/70. Sats 94% on 2L • Murmur...
Investigations • CxR: Moderate cardiomegaly with pulmonary vascular congestion. • BNP 1453. • Pt was started on aspirin, clopidogrel, metoprolol, ramipril, atorvastatin and therapeutic clexane. • TTE performed.
Progress • Pt was placed on the list for transfer to St. Vincents for surgery. • Angiogram • Wait seemed to be forever. • Pt made it to Sydney. TTE and TOE done. • Pt died suddenly on the weekend while waiting for surgery the next week.
VSD complicating MI • Typically 3-5/7 post mi (hrs-weeks). May occur a bit earlier with fibrinolytics. • RFs: Single vessel disease (esp LAD and wrap around LADs), extensive myocardial damage, poor septal collateral supply. • Rate of 0.2% in the GUSTO trial (higher with conservative Tx). • Rupture occurs at the margin of the necrotic and non-necrotic tissue. (1)
Presentation • Presentation is usually with sudden onset of haemodynamic compromise, bi-ventricular failure (often R>L) and a new murmur. • In a TTE study of 43 pts who had VSDs, the defect could be seen in 40% on 2D alone and in 100% with the use of colour doppler. • Management: Stabilisation can be attempted with vasodilators, inotropic agents, diuretics and IABP counter-pulsation. (1)
Management • In GUSTO-1 40% of pts with a VSD underwent surgical repair (median 3.5/7). • Survival at 30 days was 53 vs 6% and at 1 year 47 vs 3% without surgery. • Delayed repair in those without shock is possible but rapid deterioration may be unpredictable. • Advanced age and longer delay to surgery are associated with poorer outcomes. (1)
Management • Long term survival is increased by bypassing associated coronary disease. Survival of 72 vs 48% at five years in one trial. • Determinants of late cardiac mortality were post op cardiac failure, incomplete revascularisation and longer aortic cross clamp time. • Trans-catheter closure. (1)
Case 3 • 72 yo female presented referred from gp with around 12/12 of a hoarse voice, progressive dysphagia and neck swelling. • Worse over the last 3/12. • Increasing peripheral oedema and increasing SoB over the last few months. • Seen by ENT as an OP: atrophied vocal cord. • PHx: GORD, mild asthma.
Case 3 • O/E: Swollen lips and tongue noted, no lymphadenopathy and a tethered submandibular mass was felt. • CT: Oesophageal oedema, no clear submandibular mass seen, artefact from amalgum fillings. • gastroscopy: Gross infiltration of the base of the tongue with venous congestion of the tongue and oesophagus, atrophic vocal cord and no mucosal abnormality.
Case 3 • Pt was transferred to Tamara for a tongue biopsy. • Biopsy: Sections show green birefringence under polarised light with congo red stains. • Confirming amyloid deposits. Immunostains proceeding to characterise the type of amyloid. • Pathology: FBC and UEC unremarkable. BNP 34 (N <20). • Paraprotein identified as monoclonal free lambda light chains on serum and urine.
Case 3 • Pt transferred back to Tamworth hospital now. More SoB than previous.
Case 3 • Pleural effusion was drained and diuretics commenced with good result. • Discussion with haematology and colchicine was started. • View to haematology O/P follow up.
Case 4 • 76yo man referred to hospital by GP for cellulitis of his hand. • Rheumatoid arthritis for probably 15 yrs with poor treatment. Used sulfasalazine in the past without much effect and naproxen and fish oil lately with some pain relief. • Cardiac Hx...
cardiac Hx • Collapse and broad complex tachycardia 5 yrs ago. • EPS and review with Dr. Barlow resulted in an ICD being implanted. • DCCV for atrial flutter. • TTE 2-3 years ago showed marked left ventricular hypertrophy so pt was sent to sydney for a biopsy.
progress • This showed AA amyloidosis. No further follow up occurred. • On this admission pt developed interstitial nephritis from cephazolin and worsening heart failure which responded to diuretics. • He was reviewed by haematology and a work up for haematological malignancy was performed and he was also started on colchicine with o/p f/u.
Amyloidosis • Extracellular tissue deposition of fibrils composed of low molecular weight subunits of various serum proteins that adopt a B pleated sheet configuration. • AL amyloidosis (a plasma cell dyscrasia) where the amyloid protein is composed of a mono-clonal light chain. • AA amyloid is due to fragments of serum amyloid A protein (an acute phase reactant) associated with chronic inflammatory disease and rarely produces clinically apparent heart disease. (2)
Amyloidosis • Amyloidosis can also occur due to a transthyretin mutation (a small protein produced by the liver) that is AD with high penetrance. • Clinical evidence of cardiac involvement occurs in ~50% of AL patients. • It often leads to significantly rapid and progressive cardiac dysfunction. • Median survival in one study was 11 months with AL amyloid vs 75 months in AA amyloid. (2)
AL Amyloidosis • Is a multi-system disorder. Cardiac amyloid is rarely the sole manifestation (4%). • Clinical picture is dominated by right heart failure (APO is rare). Some pts get angina with involvement usually of small and intra-myocardial coronaries. • Postural hypotension is common, ventricular arrhythmias are often poorly tolerated and fatal and high degree AV block is unusual despite conduction system involvement. • Asymmetric septal hypertrophy mimicking hocm is rare. (More common in familial amyloidosis). (2)
TTE • LVH and evidence of diastolic dysfunction are the earliest abnormalities. • In more advanced disease, wall thickening progresses resulting in restrictive CM. Bi-atrial enlargement occurs, the RV may dilate and MV and AV leaflets become thickened. A restrictive filling pattern is seen on diastology. • Only 26% of pts had granular sparkling myocardium in one series. • There is marked dissociation between short and long axis systolic LV function. • Intra-cardiac thrombus is common (~27%), usually in the LA appendage. (2)