Case submission for CSI Asia-Pacific 2018- Case 2 Title Page Case category: Coarctation and ducts, valves Title: Simultaneous balloon aortic valvuloplasty with transcatheter closure of large hypertensive patent ductus arteriosus in a young child Authors: 1. Girish R Sabnis MD, DM * 2. Charan P Lanjewar MD, DM, FACC 3. Hetan C Shah MD, DNB 4. Prafulla G Kerkar MD, DM, FACC Affiliation for all authors: Department of Cardiology, Seth G.S. Medical College and King Edward Memorial Hospital, Parel, Mumbai, India *Corresponding author Address: Department of Cardiology, Room number 403, 4th floor, C.V.T.C. Building, K.E.M. Hospital, Parel, Mumbai- 400012, MH-INDIA email: girishsabnis@live.in Mobile: +919833712374 Phone: +912224107636
History and physical: A 10-years-old male child presented with worsening effort tolerance and increasing frequency of syncopal episodes since last two months. He had been diagnosed with congenital heart disease in infancy and advised surgery which was declined due to financial constraints. Presently, the referring physician had deemed the problem as inoperable and referred for medical management. On examination, the child showed evidence of growth retardation and weighed 17 kg. The pulse was 96 beats per minute and appeared to be of normal volume. The SpO2 was 98% in both the upper limbs and 96% in both the lower limbs. Precordial palpation revealed cardiomegaly, prominent right ventricular heave and a thrill in the suprasternal notch. On ausculatation, there was a harsh grade IV ejection systolic murmur in the aortic area radiating to the carotid arteries. Another predominantly systolic murmur was appreciated in the left second intercostal space. The ECG revealed sinus rhythm, rightward QRS axis and biventricular enlargement. Chest X-ray showed cardiomegaly with prominently enlarged branch pulmonary arteries. Imaging: Echocardiography revealed prominent enlargement of the left chambers along with concentric LV hypertrophy and good biventricular function. Aortic valve was bicuspid with very severe stenosis ( jet velocity 5 m/s, mean gradient of up to 60 mm Hg, annulus size 18 mm) and trivial regurgitation. A large (6.5 mm) PDA (figure 1) was demonstrated with predominantly systolic left-to- right shunting, dilated PAs and doppler evidence of severe PAH. Indication for intervention: Very severe aortic valve stenosis with hypertensive PDA Intervention: Procedure was performed under sedation. Accesses: 7 Fr right CFA, 6 Fr right CFV, 4 Fr left CFA (for pressure monitoring). The PA pressures measured with a 5 Fr JR catheter were near systemic and the measured Qp:Qs was 2.1. There was significant fall in the PA pressure and the PVR along with rise in the aortic pressure on administration of 100% oxygen. DTA angiogram in the left lateral view showed a large 7 mm
tubular ductus (figure 2). Ascending aortography was performed with 5 Fr pigtail catheter in the same view and the stenotic annulus demonstrated. The valve was crossed using a straight tip 0.035 Terumo glide wire and 6 Fr AL1 catheter. Peak-to-peak transaortic gradient of 80 mm Hg was demonstrated. The wire was exchanged with an exchange length 0.035 J-tip guide wire and a 17 mm x 40 mm Tyshak balloon was positioned across the valve and inflated under rapid atrial pacing till the waist gave way (figure 3). There was no residual transaortic gradient and more than mild regurgitation in the repeat aortogram. The duct was crossed from the PA with an exchange length 0.035 straight tip guide wire and the JR was exchanged with a 7 Fr Amplatzer TorqVue delivery system. A 10 mm Amplatzer muscular VSD occluder device was positioned across the duct and a significant fall in PA pressure and rise in aortic pressure was demonstrated before releasing the device from its cable. Final angiogram revealed good device position and no residual shunt (figure 4). Procedure and further ward course was uneventful. The patient is symptom-free at 6 months follow-up. Fig 1
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Learning points: 1. Importance of clinical judgement in assessment of hypertensive ducts and associated lesions. 2. Contrary to the usual teaching of tackling the distal lesion first, the aortic valve was treated before the PDA. The rationale was to enable accurate hemodynamic assessment of the duct and to prevent device instability due to sudden increase in aortic pressure after relief of stenosis. 3. The utility of double-disk devices in the closure of large hypertensive ducts.