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Heart, Lung and Circulation (2015) xx, 1–4 1443-9506/04/$36.00 http://dx.doi.org/10.1016/j.hlc.2015.06.820
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Headache in a Patient with Complex Congenital Heart Disease: Diagnostic and Therapeutic Considerations
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[TD$FIRSNAME]Waheed[TD$FIRSNAME.] [TD$SURNAME]Ahmad[,TD$SURNAME.] MBBS, [TD$FIRSNAME]Ferdi[TD$FIRSNAME.] [TD$SURNAME]Miteff[TD$SURNAME.], FRACP, [TD$FIRSNAME]Nicholas[TD$FIRSNAME.] [TD$SURNAME]Collins[TD$SURNAME.], FRACP *
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CLINICAL SPOTLIGHT
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Cardiovascular Unit, John Hunter Hospital, Newcastle, NSW Australia, 2305 Received 28 February 2015; received in revised form 29 May 2015; accepted 6 June 2015; online published-ahead-of-print xxx
Headache in adult patients with congenital heart disease may be a manifestation of the underlying cardiac condition or more common alternative causes of headache. In patients with pre-existing congenital heart disease, consideration of potentially uncommon aetiologies of headache is important. We report an uncommon case of headache in a patient with complex congenital heart disease characterised by Ebstein’s anomaly with previous surgical repair complicated by idiopathic intracranial hypertension. This case illustrates the importance of understanding the implications of headache with reference to the underlying cardiac disease as well as specific issues related to a relatively young cohort of patients. Keywords
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Ebstein’s anomaly Idiopathic intracranial hypertension Glenn shunt Tricuspid regurgitation
Headache in adult patients with congenital heart disease may complicate their specific underlying cardiac condition or be a result of the myriad of more common alternative causes of headache. In patients with headache and pre-existing congenital heart disease, consideration of potentially uncommon aetiologies is important, such as hypertension in a patient with previous aortic coarctation repair, cerebral abscess in a patient with Eisenmenger’s syndrome or the reported association between migraine and intracardiac shunt. We report an uncommon cause of headache in a patient with complex congenital heart disease, in which a 23-yearold woman with a background of Ebstein’s anomaly and previous bidirectional Glenn shunt developed idiopathic intracranial hypertension. This case illustrates the importance of understanding the implications of headache with reference to the underlying cardiac disease as well as specific issues related to a relatively young cohort of patients. The patient was diagnosed with Ebstein’s anomaly and atrial septal defect in infancy and was initially palliated with a right Blalock shunt. At the age of five years, she subsequently underwent tricuspid valve repair using Carpentier
technique with plication of the right atrium and ventricle with suture closure of the atrial septal defect. Due to ongoing effort intolerance with associated severe tricuspid incompetence, right atrial and right ventricular dilatation, further surgery was undertaken at age 19. Diagnostic cardiac catheterisation prior to surgery demonstrated a low transpulmonary gradient and low pulmonary vascular resistance (mean pulmonary artery pressure 9 mmHg, mean right atrial pressure 6 mmHg, left ventricular end diastolic pressure 6 mmHg, transpulmonary gradient 3 mmHg, pulmonary vascular resistance 0.8 Woods units). The patient then underwent tricuspid valve repair, tricuspid valve annuloplasty (31 mm Duran band), right atrial reduction and bidirectional superior cavopulmonary (Glenn) shunt creation. The patient remained limited by exertional fatigue and dyspnoea following her last corrective procedure, which were attributed to severe residual tricuspid regurgitation (Figure 1). Cardiopulmonary exercise testing confirmed exertional hypoxaemia, with pulmonary embolism and pulmonary AV malformation subsequently excluded. These symptoms were effectively managed by diuretic therapy. The patient’s cardiac status
*Corresponding author at: Cardiovascular Unit, John Hunter Hospital, Newcastle, NSW Australia, 2305. Tel.: +61249214277; Fax: +61249214210, Email:
[email protected] © 2015 Published by Elsevier Inc on behalf of Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ).
Please cite this article in press as: Ahmad W, et al. Headache in a Patient with Complex Congenital Heart Disease: Diagnostic and Therapeutic Considerations. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.06.820
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Figure 1 Apical four-chamber images demonstrating severe apical tricuspid valve displacement consistent with known Ebstein’s anomaly (Figures 1a and 1b). There is severe tricuspid valve incompetence as noted in Figure 1c.
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subsequently remained stable apart from the development of atrial arrhythmia, which was managed by successful catheter ablation. The patient was maintained on no regular medical therapy aside from contraception in the form of Implanon1 (etonogestrel), a progesterone-based implantable contraceptive, which delivered subcutaneously provides up to three years of contraception. The patient then presented to emergency department with a two-week history of headache, visual disturbance and tinnitus. The patient denied diplopia. Clinical examination was unremarkable for infective or inflammatory signs. Visual acuity was reduced bilaterally. Visual fields were restricted and blind spots were enlarged. Fundoscopy revealed marked oedema of the right optic disc and mild oedema of the left disc. The remainder of her cranial nerves and limb examination were normal. Lumbar puncture revealed raised cerebrospinal fluid (CSF) pressure at 33.5 cm. Lab examination of the CSF fluid showed normal cells and protein count.
MRI brain (Figure 2) showed cupping of the optic discs and enlargement of surrounding CSF with flattening of pituitary gland consistent with raised intracranial pressure. MR venography revealed bilateral transverse sinus stenosis. Echocardiography again demonstrated diminutive right ventricular size with severe tricuspid incompetence. Flow within the superior vena cava was phasic with antegrade flow noted from the right ventricle into the main pulmonary artery; there had been no interval change in the patient’s echocardiographic appearance. Following subsequent formal neurology review, the patient was commenced on acetazolamide 250 mg three times a day. Following three weeks of therapy, the patient noted a dramatic symptomatic improvement with improved results on visual filed testing. Management was complicated, however, by medication side-effects including nausea, vomiting and paraesthesia. Due to problematic sideeffects, the acetazolamide dose was halved with return of symptoms of intracranial hypertension. Topiramate was
Please cite this article in press as: Ahmad W, et al. Headache in a Patient with Complex Congenital Heart Disease: Diagnostic and Therapeutic Considerations. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.06.820
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Headache and complex CHD
Figure 2 There is cupping of the optic discs (white arrow) and tortuosity of the optic nerves (black arrowhead) with enlargement of the surrounding CSF (Figure 2a). On the sagittal images, note is also made of flattening of the pituitary gland within the pituitary fossa (white arrowhead) consistent with raised intracranial pressure. Transverse sinus narrowing (black arrows) was noted (Figure 2b).
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added to the treatment at a dose of 25 mg per day, which was gradually increased to 100 mg a day. This was complicated by depression, worsening of headache, visual symptoms and deterioration in her visual fields. Additional treatment options included therapeutic lumbar puncture or percutaneous intervention of the previously
documented transverse sinus stenosis. Cerebral angiography and manometry of the transverse sinuses excluded a significant gradient and medical therapy was therefore continued. At this stage, the progesterone implant (Implanon1) was removed which resulted in marked improvement in her symptoms, including headache, with resolution of her
Please cite this article in press as: Ahmad W, et al. Headache in a Patient with Complex Congenital Heart Disease: Diagnostic and Therapeutic Considerations. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.06.820
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papilloedema and visual field defects. Acetazolamide and topiramate were gradually reduced and ceased as a result of the striking improvement in symptoms. Ebstein’s anomaly is an uncommon congenital cardiac condition with an estimated incidence of 1 per 200 000 live births and accounting for <1% of all cases of congenital heart disease [1]. The clinical presentation may be variable, from neonatal cardiac instability requiring intervention to detection of tricuspid valve abnormalities in an asymptomatic adult. In this case, the patient had undergone several procedures to repair the tricuspid valve previously with the additional creation of a Glenn shunt (an anastomosis between the superior vena cava and the right pulmonary artery), designed to limit the volume load placed upon the inadequately sized right ventricle and regurgitant tricuspid valve. To ensure adequate pulmonary flow, the pulmonary vascular resistance must be sufficiently low to provide an appropriate gradient for flow; this requirement is partially offset by flow through the right heart in this situation. Complications of cavopulmonary anastomosis include the development of pulmonary vascular disease, pulmonary arteriovenous malformations with concomitant cyanosis, stenosis of pulmonary artery branches and/or cavopulmonary anastomosis and recanalisation of hemiazygos vein [2]. Uncommonly described after the creation of cavopulmonary anastomosis are complications related to elevated right-sided filling pressures, which include idiopathic intracranial hypertension and hydrocephalus [2,3]. Physicians caring for patients with complex congenital heart disease should be alert to the consequences of elevated venous pressure and assess the cardiac status in patients with headache, particularly with features suggestive of idiopathic intracranial hypertension. Idiopathic intracranial hypertension refers to a condition characterised by elevated pressure within the cerebrospinal space and typically presents with headache, tinnitus and visual disturbance. Potential aetiologies include various medications, including contraceptive agents. In this unusual case, transverse venous sinus stenosis, pulsatile flow into the cavopulmonary shunt and elevated right sided filling pressures from the cavopulmonary shunt may have been
contributory to development of headache and intracranial hypertension. Given the deleterious effects of pregnancy on certain congenital cardiac diseases, contraception is frequently employed, with choice of agent influenced by the nature of residual cardiac sequelae. Patients with high risk of venous thrombosis (eg Fontan circulation) should avoid oestrogen-containing preparations; similarly patients with intracardiac shunts may be at risk of paradoxical emboli in the event of venous thrombosis. Implantable progesterone based contraceptive agents, such as Implanon1, are valuable in that they provide predictable, effective contraceptive and lack a tendency to thrombosis as is seen with oestrogencontaining contraceptives. Furthermore, implantable preparations may avoid issues related to non-compliance, which may be problematic with alternate progesterone containing compounds. While side-effects of Implanon1 are infrequent, they include headache, weight gain, dysmenorrhoea, acne, local implant site complications and depression. Idiopathic intracranial hypertension is an uncommonly described sideeffect of the use of contraceptive agents [4]. While headache may be unrelated to the underlying cardiac disease, an understanding of the congenital anomalies may broaden the differential diagnosis. Similarly, an appreciation of medical therapy used in this population may further refine the diagnostic possibilities. This case highlights the need for the consideration and thorough understanding of any underlying congenital cardiac disease in patients presenting with headache.
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[1] Brickner ME, Hillis LD, Lange RA. Congenital heart disease in adults. Second of two parts. N Engl J Med 2000;342:334–42. [2] Rosman NP, Shands KN. Hydrocephalus caused by increased intracranial venous pressure: A clinicopathological study. Annals of Neurology 1978;3:445–50. [3] Fuchs H, Singh D, Greene Jr C, Ross-Ascuitto N, Ascuitto R. Pseudotumor cerebri associated with modified fontan anatomy. Pediatric Cardiology 2013;34:1932–4. [4] Singh K, Chye GC. Adverse effects associated with contraceptive implants: Incidence, prevention and management. Advances in Contraception: the official journal of the Society for the Advancement of Contraception 1998;14:1–13.
Please cite this article in press as: Ahmad W, et al. Headache in a Patient with Complex Congenital Heart Disease: Diagnostic and Therapeutic Considerations. Heart, Lung and Circulation (2015), http://dx.doi.org/10.1016/j.hlc.2015.06.820
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