1445Ann Thorac Surg CASE REPORT RADHAKRISHNAN ET AL2014;98:1445–7 MODIFIED BENTALL IN A LAUBRY PEZZI

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Cardiovascular surgical procedures with CPB have beenreported to constitute an especially high risk for patientswith MMD because of decreasing cerebral perfusionpressure owing to the perfusion pressure variability in theinitial stages of CPB and nonpulsatile (continuous) flow[2–5]. Therefore, it is a challenging operation and requiresstrict intraoperative management, including the mainte-nance of blood pressure, hematocrit, mean arterial pres-sure, and body temperature in patients with MMD [2–5].Furthermore, to maintain pulse pressure, the use of anintraaortic balloon pump in coronary artery bypass graft-ing was reported [6].

TAVR is a therapy developed for severe aortic stenosis inhigh-surgical risk patients [7, 8]. Surgeons must keep inmind that major complications can occur at any step dur-ing TAVR, and we should prepare for fast cannulation toperform CPB. Particularly in patients with MMD, weroutinely secure not only one artery and one vein for im-mediate CPB but also one additional artery for IABPif nonpulsatile (continuous) flow occurs because of intra-operative complications. We performed TAVR in 3 pa-tients with aortic valve stenosis complicated withMMD and did not observe additional neurologic events.Although CPB was not required in the other 2 patients, inthis case, unfortunately, the hemodynamics collapsed afterballoon valvuloplasty, andCPBwas required immediately.Still, IABP was not necessary because the hemodynamicsrapidly improved as soon as CPB was started, and thepatient’s pulse pressure was well preserved during theCPB support.

The use of CPB and cardiac arrest for TAVR is needed asignificant minority of cases, and therefore, this proceduremay be a useful treatment for patients with aortic valvestenosis complicated with severe cerebral ischemia,including Moyamoya disease.

ConclusionWe successfully performed TAVR in patients who hadsevere aortic valve stenosis complicated with Moyamoyadisease. TAVR is a very useful treatment for patientswith aortic valve stenosis complicated with Moyamoyadisease.

Accepted for publication Nov 19, 2013.

Address correspondence to Dr Radhakrishnan, Department of Cardio-vascular and Thoracic Surgery, Sree Chitra Tirunal Institute for MedicalSciences and Technology, Thiruvananthapuram-695011, Kerala, India;e-mail: bineeshkrishna@gmail.com.

References

1. Suzuki J, Takaku A. Cerebrovascular “moyamoya” disease:Disease showing abnormal net-like vessels in base of brain.Arch Neurol 1969;20:288–99.

2. Han SH, Kim SD, Ham BM, et al. Normothermic cardiopul-monary bypass in a patient with moyamoya disease.J Cardiothorac Vasc Anesth 2005;19:499–500.

3. Gatti G, Benussi B, Gon L, et al. Aortic valve replacementin an adult white male with moyamoya disease and coro-nary artery fistulas. J Cardiothorac Vasc Anesth 2007;21:166–8.

4. AI-Naimi KT, Mediratta NK, Pennefather SH. Hypothermiccardiopulmonary bypass in a patient with moyamoya disease.J Cardiothorac Vasc Anesth 2009;23:206–7.

5. Wang N, Kuluz J, Barron N, et al. Cardiopulmonary bypass ina patient with moyamoya disease. Anesth Analg 1997;84:1160–3.

6. Kashima I, Inoue Y, Takahashi R. The use of intra-aorticballoon pump as cerebral protection in a patient with

� 2014 by The Society of Thoracic SurgeonsPublished by Elsevier

moyamoya disease undergoing coronary artery bypass graft-ing. Interact Cardiovasc Thorac Surg 2008;7:522–3.

7. Leon MB, Smith CR, Mack M, et al. Transcatheter aortic-valveimplantation for aortic stenosis in patients who cannot un-dergo surgery. N Engl J Med 2010;363:1597–607.

8. Smith CR, Leon MB, Mack MJ, et al. Transcatheter versussurgical aortic-valve replacement in high-risk patients. N EnglJ Med 2011;364:2187–98.

Primary Modified Bentall’sProcedure in a Case ofLaubry-Pezzi SyndromeBineesh K. Radhakrishnan, MCh,Mohammed Idhrees A., MS, Sujith Devarajan, MS,Varghese T. Panicker, MCh, Vivek V. Pillai, MCh,Praveen K. Varma, MCh, andJayakumar Karunakaran, MCh

Department of Cardiovascular and Thoracic Surgery, Sree ChitraTirunal Institute for Medical Sciences and Technology,Thiruvananthapuram, Kerala, India

Modified Bentall’s procedure done as part of the primaryrepair in Laubry-Pezzi syndrome is very rarely describedin the literature. We present a case of a 33-year-old manwith a subpulmonic venticular septal defect, aneurysmaldilatation of the aortic root and ascending aorta, with anassociated patent ductus arteriosus, corrected by theincorporation of Yacoub’s techique for ventricular septaldefect closure with a modified Bentall’s procedure andtranspulmonary patent ductus arteriosus ligation. Thepostoperative course was unremarkable. Early follow-upreports show good biventricular function without resid-ual ventricular septal defect or iatrogenic ventricularoutflow tract obstructions.

(Ann Thorac Surg 2014;98:1445–7)� 2014 by The Society of Thoracic Surgeons

harles Laubry and Cesare Pezzi described the clin-

Cical features of Laubry-Pezzi syndrome in 1921 in apatient with ventricular septal defect (VSD) with aorticregurgitation caused by aortic valve prolapse [1]. Thesyndrome may eventually lead to dilatation of the aorticsinus related to the involved leaflet, resulting from a lackof continuity of the aortic media and annulus [2, 3].It may also be possible that, as in the case of various

other pathologic conditions like Marfan’s syndrome andbicuspid aortic valve, the ascending aorta may also beinvolved structurally in the natural history of the disease[2]. However, it is very rare for these patients to progressto aneurysmal dilatation of the aortic root and ascendingaorta at the time of a first operation.

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Fig 2. A view through the open aorta, after excision of valve leaflets.Right and left ventricles are seen communicating at the septal defect.Right and left coronary buttons can be seen.

1446 CASE REPORT RADHAKRISHNAN ET AL Ann Thorac SurgMODIFIED BENTALL IN A LAUBRY PEZZI 2014;98:1445–7

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A 33-year-old man with symptoms and signs of severeaortic insufficiency (New York Heart Association class III)was referred to us for an elective operation fromthe cardiology department of our hospital. His echocar-diographic reports were consistent with a 0.5-cm sub-pulmonic VSD jet, with prolapse of the right coronarycusp into the VSD, thereby partly closing the defect andproducing severe aortic regurgitation. The left ventriclewas dilated, with an end-diastolic diameter of 9.5 cm andan ejection fraction of 0.44. A right ventricular outflowtract gradient of 30 mm Hg was noted, and a 0.5-cmpatent ductus arteriosus (PDA) was also found, withmoderate pulmonary artery hypertension. The aorticroot and ascending aorta were aneurysmally dilated.Computed tomography angiography showed aortic rootdimensions of 6.3 � 6.1 cm and sinotubular junction di-mensions of 5.6 � 5.5 cm. The ascending aorta measured5.5 cm (Fig 1). Considering the patient’s age and the long-standing nature of the right coronary cusp prolapse,which might have caused structural abnormalities of thecusp, with severe aortic regurgitation, we planned toproceed with a modified Bentall’s procedure and closureof the VSD and PDA.

The operation was performed through a median ster-notomy, with the patient under cardiopulmonary bypasswith aortic cross-clamping, under moderate hypothermia.Transpulmonary closure of the PDA was done withpledgetted 4-0 polypropylene suture. For the VSD closure,which was 5� 5 cm (Fig 2), we used Yacoub’s technique. Inthe area of the right coronary sinus, horizontal mattresssutures with pledgetted 2-0 polytetrafluroethylene werepassed from the crest of the interventricular septum andbrought out through the aortic annulus, which on pullingup approximated the crest with the aortic annulus, therebyclosing the VSD. In the other areas, everting annular

Fig 1. Computed tomogram showing aortic root and ascending aorticaneurysm.

sutures were taken (Fig 3). A valved conduit prepared onthe table, with use of a No. 29 tilting disc valve and a no. 30albumin-coated polyester graft, was seated onto theannulus by use of these sutures and secured. The left andright coronary buttons were anastomosed to the graft with5-0 polypropylene sutures. A distal anastomosis wasperformed with the distal ascending aorta by use of 3-0polypropylene sutures. The aortic cross-clamp wasremoved after meticulous deairing, and the patient wasweaned from cardiopulmonary bypass, with minimalinotropic support of adrenaline 0.05 mg/kg�1/min�1. Hispostoperative recovery was unremarkable, and he wasdischarged on postoperative day 7.Echocardiography at discharge showed no residual

VSD or PDA, good biventricular function, and a mildright ventricular outflow tract gradient (which was pre-sent preoperatively also and was attributed to a promi-nent muscle bundle in the right ventricle). The leftventricular end-diastolic diameter decreased to 7 cm. Atthe 6-month follow–up visit, he had good biventricularfunction, without progression of the right ventriculargradient, as shown by echocardiography.

Comment

Knowledge of the natural history of Laubry-Pezzi syn-drome is limited in the literature because of the earlyintervention recommended in the form of VSD closurewith or without aortic valve repair or replacement [4]. Inthe later stages of the disease, the hemodynamics are

Fig 3. Ventricular septal defect undergoing closure by use of Yacoub’stechnique.

Accepted for publication Dec 9, 2013.

Address correspondence to Dr Roubelakis, Department of CardiothoracicSurgery, Southampton University Hospital, Tremona Road, Southampton,SO16 6YD, United Kingdom; e-mail: e-roube@hotmail.com.

1447Ann Thorac Surg CASE REPORT ROUBELAKIS ET AL2014;98:1447–9 TRICUSPID VALVE REPAIR USING NEOCHORDS

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governed by the volume overload of the left ventriclecaused by aortic incompetence rather than the shuntacross the VSD [4, 5]. Owing to a possible structural ab-normality in the sinus and in the ascending aorta, aneu-rysmal dilatation of the aortic root and ascending aorta ispossible in the later stages of the disease [2, 3]. Yacoub’stechnique for VSD closure [6] can safely be considered insuch cases, without concerns about tension on the sutureline, because of the redundancy of tissues in the ventric-ular aspect. To the best of our knowledge, this is the firstcase ever reported in the literature: performance of amodified Bentall’s procedure incorporating Yacoub’stechnique of VSD Closure as a part of the primary oper-ation in a case of Laubry-Pezzi syndrome [2] and probablythe first case to combine a modified Bentall’s procedure,VSD closure without a patch, and PDA closure.

References

1. Laubry C, Pezzi C. Trait�e des maladies cong�enitales du coeur.Paris: JB Bailliere; 1921. Quoted by Labury C, Routier D,Soulie P. Les souffles de la maladie de Roger. Rev Med Paris1933;50:439–448.

2. Martınez-Quintana E, Rodrıguez-Gonzalez F, L�opez-Gude MJ. Laubry-Pezzi syndrome with aortic root dilatationtreated with a Bentall and De Bono procedure. World J PediatCongenit Heart Surg 2013;4:299–301.

3. Sakakibara S, Konno S. Congenital aneurysm of the sinus deValsalva associated with ventricular septal defect: anatomicalaspects. Am Heart J 1968;75:595–603.

� 2014 by The Society of Thoracic SurgeonsPublished by Elsevier

4. Ammash M, Warnes CA. Ventricular septal defects in adults.Ann Intern Med 2001;135:812–24.

5. Nadas AS, Thilenius OG, Lafarge CG, Hauck AJ. Ventricularseptal defect with aortic regurgitation: medical and pathologicaspects. Circulation 1964;29:862–73.

6. YacoubMH, KhanH, Stavri G, Shinebourne E, Radley-Smith R.Anatomic correction of the syndrome of prolapsing right coro-nary cusp, dilatation of the sinus of Valsalva and ventricularseptal defect. J Thorac Cardiovasc Surg 1997;113:253–60.

Isolated Tricuspid Valve RepairAfter Metastatic Tumor ResectionApostolos Roubelakis, PhD, Amit Modi, FRCS (C-Th),and Sunil K. Ohri, MD, FRCS (C-Th)

Department of Cardiothoracic Surgery, SouthamptonUniversity Hospitals NHS Foundation Trust, Southampton,United Kingdom

Isolated tricuspid valve repairs are performed in adultsmost commonly as a result of infective endocarditis,traumatic injury, rheumatic involvement, and ischemia.The use of neochordaemade from polytetrafluoroethylene,being widespread in mitral valve operations, is gainingground in tricuspid valve repair. We report a very rare casethat describes isolated tricuspid valve repair using neo-chordae after resection of a right ventricular tumorinvolving the tricuspid valve. The tumor isolated wasmetastatic teratoma in a patient with growing teratomasyndrome. We further describe our technique of tricuspidneochord implantation and the rationale behind the repair.

(Ann Thorac Surg 2014;98:1447–9)� 2014 by The Society of Thoracic Surgeons

e present a case of an adult patient with a diagnosis

Wof growing teratoma syndrome, which involved theright ventricle and the tricuspid valve. For the excision ofthe tumor, tendinous chords of the anterior leaflet had tobe excised. The tricuspid valve was repaired with artificialpolytetrafluoroethylene (PTFE) neochordae. Few reportshave been published presenting the above repair tech-nique for the tricuspid valve, but in this report thistechnique is being used after tumor resection.

A 25-year-old patient received a diagnosis of growingteratoma syndrome after neoadjuvant chemotherapy, leftorchidectomy, and excision of a para-aortic lymph nodalmass and an inferior vena caval mass. During the opera-tion, a transesophageal echocardiography (TEE) probewas deployed to identify the proximal limit of the inferiorvena caval tumor extension. This scan revealed a pedun-culated mobile mass of unknown origin in the rightventricle. Cardiac magnetic resonance imaging (CMR)and transthoracic echocardiography (TTE) elucidated thepossible causes in the postoperative period. TTE revealed a

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