It is a report of an unusual case of recurrent periprosthetic aortic regurgitation which occurred four times in the same patient over a span of 26 years. After pre-operative stabilization, the patient was subjected to a fifth open heart surgery to replace the 4th prosthetic aortic valve by an upsized 5th prosthetic valve. Since endocarditis was excluded by repeated blood culture and paucity of vegetations, a working diagnosis of Behcet's disease was arrived upon. The fifth prosthetic aortic valve was implanted using a special suturing technique as described by Azumo, et al in 2009, which involves a sub-annular reinforcement by a Teflon ring. The patient was discharged after an uneventful recovery and is well on regular follow-up.

1. Fourth Recurrence of Aortic Annular Dehiscence
Following AVR for Aortic Regurgitation

2. Case Report
Though not common, peri-prosthetic dehiscence
following heart valve surgery occurs in about 2% cases and
mostly this is due to endocarditis involving the annulus.
Generally in all this situation, needs is resuturing of the
dehisced prosthesis or replacement of infected prosthesis
to the native annulus preceded and followed by
appropriate antibiotic therapy.
Rarely, prosthetic heart valve dysfunction could result
in prosthetic or bioprosthetic trans-valvular regurgitation
and this could necessitate re-replacement of the said valve
(aortic or mitral).
The following is a case report of patient who presented
with aortic regurgitation to Christian Medical College,
Vellore in 1985. He underwent aortic valve replacement
(AVR) with caged ball prosthesis (Starr Edward 9A). He
had uneventful recovery and was discharged after the (than
usual) hospital stay of a fortnight.
He presented to the authors for the first time to Batra
Hospital, New Delhi with severe para-valvular aortic
regurgitation (AR). A mono leaflet tilting disc prosthesis
(Medtronic 23A) for re-implantation (Table 1).
Subsequently, eleven years after the second AVR he
presented to the same team at the Indraprastha Apollo
Hospital with significant mitral valve regurgitation and
mild para-valvular AR. There was a recent history of fever
and therefore he received 2 weeks of antibiotic before third
time double valve replacement (DVR). A third valve
FOURTH RECURRENCE OF AORTIC ANNULAR DEHISCENCE
FOLLOWING AVR FOR AORTIC REGURGITATION
Ganesh K Mani, Jeet Ram Kanwar, Pramod Kumar Sharma, Ajeet Jain, Sri Krishan Gupta
and Manju Mani
Department of CTVS, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi 110 076, India.
Correspondence to: Dr Ganesh K Mani, Senior Consultant Cardiac Surgeon, Department of CTVS, Indraprastha
Apollo Hospitals, Sarita Vihar, New Delhi 110 076, India.
E-mail: gkm@ganeshmani.com
It is a report of an unusual case of recurrent periprosthetic aortic regurgitation which occurred four times in the
same patient over a span of 26 years. After pre-operative stabilization, the patient was subjected to a fifth open
heart surgery to replace the 4th prosthetic aortic valve by an upsized 5th prosthetic valve. Since endocarditis
was excluded by repeated blood culture and paucity of vegetations, a working diagnosis of Behcet’s disease
was arrived upon. The fifth prosthetic aortic valve was implanted using a special suturing technique as
described by Azumo, et al in 2009, which involves a sub-annular reinforcement by a Teflon ring. The patient
was discharged after an uneventful recovery and is well on regular follow-up.
Key Words: Redo Aortic valve replacement, Redo open heart surgery, Behcet’s disease.
replacement was done in 2000 when the native mitral
valve was replaced with a bi-leaflet prosthesis (OnX
27M) and the mono-leaflet aortic prosthetic valve was
also replaced with bi-leaflet prosthesis (OnX 23A). Al
blood culture and explanted aortic prosthesis culture were
reported as sterile hence prolong antibiotic therapy was
not given postoperatively as would have been given in
case of infective endocarditis. He was afebrile and
remains well for next 3 years!
In 2003, he came back with severe para-valvular AR,
though mitral valve prosthesis was functioning well. He
underwent a fourth sternotomy and on aortotomy, it was
found that aortic prosthesis was intact, there were no
vegetations, but aortic annulus was separated from the
prosthesis along the half of the right coronary and the
entire non-coronary annulus. Aortic annular dilatation
was noted. Aortic prosthesis explanted and replaced with
up-sized bi-leaflet prosthesis (St Jude Medical 25A) using
interrupted, horizontal mattress, pledgeted sutures. Post-
operative course remain uneventful. Patient remained in
regular follow-up with theAuthor.
Twenty Six years after first AVR and Eight years after
fourth AVR, CKM (58 years) presented once again to the
Indraprastha Apollo Hospital in progressively increasing
congestive heart failure. As a matter of fact, he presented
with cold and clammy hands to the outpatients department
with angina, breathlessness and swollen feet! He was
rushed to the cardiac intensive care unit where he was
Apollo Medicine, Vol. 8, No. 3, September 2011 238

3. Case Report
239 Apollo Medicine, Vol. 8, No. 3, September 2011
Table 1. History of previous surgeries
Procedure Valve used Hospital Operated By Year
AVR Starr Edward 9A CMCH, Vellore SJ, et al 1985
I RedoAVR Medtronic 23A Batra HospitalNew Delhi GKM, et al 1989
II RedoAVR + MVR ON-X 23A ON-X 27M Indraprastha Apollo Hospitals. GKM, et al 2000
III RedoAVR only SJM 25A Indraprastha Apollo Hospitals. GKM, et al 2003
Table 2. Clinical features
• Pulse -98/min, Blood pressure – 96/44 mmHg (on
inotropes).
• Respiratory rate – 40/min, accessory muscular
activity +.
• JVP – raised.
• Pedal edema present
• Chest – Bilateral vesicular breathing
• B/Lbasal crepts present
• Early Diastolic murmur – Gr.IV aortic area &
parasternal
• Valve clicks – present
• Abdomen – Soft, Bowel sounds present, Liver
palpable 2 cms. Mild ascites
• CNS – Clinically NAD.
Table 3. Blood Investigations
Hematological
Hemoglobin: 10.2 g/dL TLC:10800/cmm
Platelets: 1,35000 cu cm INR: 1.3
PTTK/Control: 42.1 B. Sugar:152mg/dL
D.L.C: N-81,L-11,M-6,E-2 B. Urea- 86mg/dL
Biochemistry
Creatinine: 1.5mg/dL Sodium:141mEq/L
Potassium: 4.6m Eq/L SGOT:28
SGPT: 26 Alk. Phos: 72 U/L CPK: 8 CK- MB: 32
S. Chloride: 108mEq/L S. Protein(T) 7.4 g/dL
S. Bilirubin(T) : 0.9mg/dL S. Bilirubin(D): 0.5 mg/dL
S. Calcium: 9.2
Microbiology
Blood culture (aerobic, anaerobic & fungal) x 3 sterile
after 48 hours incubation.
treated with inotropes and non-invasive ventilation
(Tables 2 & 3, Figs. 1 & 2).
A bed side 2D Echocardiography showed a severe
paravalvular aortic regurgitation, rocking motion of the
bileaflet aortic prosthesis, normally functioning mitral
valve prosthesis with no significant gradients or
regurgitation. There were no vegetations seen in vicinity of
either prosthesis. Left ventricular ejection fraction (LVEF)
was grossly impaired to 25% and left ventricle was hugely
dilated. Because of angina like symptoms, demonstrable
coronary ischemia on ECG and age, he was taken to the
cath lab and Coronary Angiogram (CAG) and aortogram
was done with some difficulty as he was on orthopnoic and
needed non-invasive ventilation (Table 4, Fig.3).
CAG revealed normal coronaries but Fluoroscopy
showed interesting Hoola-Hoop sign, which consistent of
to and fro movement of sewing ring of prosthetic aortic
valve. This sign on fluoroscopy is pathognomic of
prosthetic valve dehiscence. Aortogram showed severe
paravalvularAR (Fig 4).
Table4. Pre-operative 2D Echo and colour Doppler
report
• Severe paravalvular aortic regurgitation from just
beyond the posterior aspect of the prosthetic stent.
• Rocking motion of the discAVR prosthesis !
• Normally functioning bileaflet MVR prosthesis with
acceptable peak and mean pressure gradients.
• Severe global LV hypokinesia of hugely dilated LV
with severely reduced global LV systolic function.
LVEF-25%.
• Good RV function.
• No LV thrombus.
• Markedly elevated LVEDP (28mmHg).
• Elevated RA pressure CVP (20mmHg).
• No MR, NoAS/AR.
• Moderate PAH.
• Mild to moderate TR (60mmHg).

4. Case Report
Apollo Medicine, Vol. 8, No. 3, September 2011 240
Fig 3. Echo showing Paravalvular leak.
Fig 4. ‘Hoola-Hoop’ sign on fluoroscopy and angiography.
Fig 5. (A and B) Aortic annulus was reinforced with
sub-annular ring. Each suture was taken as vertical
mattress suture sandwiching annulus between
prosthesis & ring [1].
Fig 1. Pre-op X ray chest showed features of pulmonary
oedema
Fig 2. ECG suggestive of Coronary Ischemia ?
• After thorough investigations and stabilization he
was taken up for the Fifth open heart surgery with a
plan to do Bentall’s procedure. Femoral artery was
exposed but not cannulated. Redo sternotomy was
done with the oscillating saw and all precautions.
Dissection of intra pericardial adhesion was done on
beating heart with help of electro-cautary and
scissors. Cardiopulmonary Bypass was established
with ascending aortic and Right Atrial cannulation.
He was cooled to Moderate hypothermia. Transverse
aortotomy was done as aorta was scarred with
previous multiple oblique aortotomy. Cardioplegic
arrest was achieved with Bretshnieder solution
preceded byAdenosin induction delivered directly to
coronary ostea (ultra-filtration used for fluid
overload). Dehisced aortic valve prosthesis was
explanted and 5th prosthetic aortic valve re-
implanted by special suture technique. This
technique was described by Azuma, et al [1]. In this
technique, a ring-shaped prosthetic graft put in sub-
annular position and sandwiched the aortic valve
annulus between the prosthetic valve and graft with
vertical mattress sutures (Fig 5).

5. Case Report
241 Apollo Medicine, Vol. 8, No. 3, September 2011
Post-operative recovery was uneventful. Heart
resumed beating in normal sinus rhythm. All post-
operative blood investigation including culture and
sensitivity came unremarkable. He remained afebrile. His
post-operative 2D Echo showed normally functioning
aortic and mitral prosthetic valves with peak and mean
pressure gradients in the normal range. There was no AR
or MR. there were moderate generalized global LV
hypokinesis with overall moderately reduced global LV
systolic function with LVEF-35%. He was discharged on
8th post operative day.
DISCUSSION
Para-valvular leak or regurgitation is not un-common
after prosthetic valve replacement surgery [2-7].
Possible Reasons for Implant Dehiscence in Any
Patient:
I. Congenital Predisposition [4]
(i) Marfans
(ii) Growing child
II. Faulty Technique [2]
(i) Inadequate anchorage by suture, Unraveled
knots?
(ii) Undersized valve
III. Endocarditis [3, 5, 6, 8]
(i) Presents as fever leucocytosis, positive cultures,
splenomegaly
(ii) Culture negative endocarditis (?) Hacek bacteria
IV. Degeneration [3]
(i) Aortic medial degeneration would cause annulo-
aortic ectasia which is marked by dilatation of
sino-tubular junction.
V. Behcet’s Disease [9-11]
(i) Rare, more common in Japanese population
Possible Cause of this fourth Recurrent Dehiscence
Q: Could it be faulty technique?
A: ‘unlikely’ as there was always an AR free period as
CKM presented after 5-6 years (average) and each time
interrupted pledgeted sutures were used by experienced
team!
Q: Could it be repeated bouts of endocarditis?
A: Unlikely as:
(i) No significant positive blood cultures seen.
(ii) No vegetation on prosthesis
(iii) Mitral valve normal
Q: Could it be under-sizing of valve?
A: Unlikely cause of dehiscence as under-sized prosthesis
will not leak only after 5 years!
Q: Could it be dissectingAneurysm?
A: There was no intimal flap / false lumen. Each time the
valve was upsized by at least one size.
Q: Could it be Behcet’s disease?
A: Perhaps Most likely, as ascending aorta and sino-
tubular function appeared normal and only annulus was
dilating progressively. Mitral valve prosthesis was not
disrupted.
By a process of systematic elimination of all
differential diagnosis, Behcet’s disease appears to be the
possible etiology in this case! (We think it is causative
factor for repeated dehiscence of aortic valve prosthesis in
this particular patient by the exclusion of other
diagnosis.).
Behcet’s disease typically dilates aortic annulus and
many a times it produces aneurysm [1, 9-12]. Behcet’s
disease is an autoimmune collagen disorder with Bizarre
presentation like aphthous ulcers, stomatitis, iridocyclites,
genital ulcers and aortic regurgitation. Progressive aortic
regurgitation was seen in 5-19% patients of Behcet’s
disease which were characterized by selective aortic
annular dilatation. In addition it may present as solitary or
multiple aneurysm in the aorta while Sino-tubular junction
may be spared!
In our fifth aortic valve replacement surgery we did use
special suturing technique as advised byAzuma [1] for the
aortic valve replacement surgery in cases of Behcet’s
disease. We believe reinforcing of aortic annulus to Teflon
felt ring towards LV side will prevent further dilatation
and the Sixth time Dehiscence!
Epilogue: Though patient is doing well clinically and
echocardiographically, immunosuppressive drugs are still
being considered assuming the diagnosis to be Behcet’s
disease.

6. Case Report
Apollo Medicine, Vol. 8, No. 3, September 2011 242
ACKNOWLEDGEMENT
The authors express their thanks to Dr Alok Kumar
Sharma for his help and support.
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