Bortolotti, Uberto

Mecozzi, Gianclaudio

Milano, Aldo

Nardi, Carmela

Pratali, Stefano

Elsevier - Publisher Connector 

Severe quadricuspid aortic valve stenosis after mediastinal irradiation
Gianclaudio Mecozzi, MD,a Stefano Pratali, MD,a Aldo Milano, MD,a Carmela Nardi, MD,b and Uberto Bortolotti, MD,a
Pisa, Italy
Quadricuspid aortic valve is an uncommon cause ofvalve dysfunction, because it is usually found at nec-ropsy or incidentally recognized at operation for se-vere aortic regurgitation.1-3 Severe aortic stenosis re-
quiring valve replacement involving a quadricuspid
aortic valve has not been reported. In the case presented here,
stenosis in a quadricuspid aortic valve is likely to have been
favored by previous mediastinal irradiation.
Clinical Summary
A 48-year-old man began having exertional angina 1 year before
admission. Risk factors for cardiovascular disease included hyper-
cholesterolemia and smoking. Approximately 30 years previously,
he had received mediastinal irradiation for a metastatic seminoma
with a total radiation dose of 3000 cCy without further sequelae. A
stress test was negative for myocardial ischemia, but a transtho-
racic 2-dimensional echocardiogram disclosed severe calcific aor-
tic stenosis with a peak transvalvular gradient of 90 mm Hg and
trivial aortic regurgitation. There was also evidence of left ven-
tricular hypertrophy with preserved function. After recurrent epi-
sodes of chest pain, he was admitted for further evaluation and
treatment. Physical examination showed a systolic ejection mur-
mur in the aortic area. The electrocardiogram disclosed sinus
rhythm and signs of left ventricular hypertrophy, whereas the chest
x-ray film showed no abnormalities. Angiography showed occlu-
sion of the right coronary artery and stenosis of a small marginal
branch of the left circumflex; fluoroscopy revealed diffuse calci-
fication of the aortic root. At operation in September 2002, calci-
fications appeared confined to the aortic root. The aortic arch was
cannulated, and on moderately hypothermic cardiopulmonary by-
pass, the heart arrested with cold blood cardioplegic solution
infused into the aortic root. A segment of saphenous vein was
grafted to the right coronary artery. The aortic valve appeared
formed by 3 cusps of equal size and a small accessory cusp
between the right and noncoronary cusps; all cusps were fibrotic
and grossly calcified (Figure 1). Because of the coarse calcification
of the aorta, the aortic valve and root were replaced with a 23-mm
cryopreserved aortic homograft with coronary ostia reimplanta-
tion. The proximal end of the venous graft was then anastomosed
to the donor aorta before release of the aortic crossclamp. The
patient recovered uneventfully and was discharged on postopera-
tive day 7.
Discussion
The adverse effects of mediastinal radiation therapy on the car-
diovascular system are well documented. Pericardial disease is
clinically evident in 60% to 70% of patients receiving mediastinal
irradiation followed by valvular and coronary disease.4 In a post-
mortem series, Brosius and colleagues5 found that 80% of the
patients studied showed evidence of valve damage after mediasti-
nal irradiation. Veinot and Edwards6 reported a pathologic study of
27 patients exhibiting radiation-related cardiac injuries involving
From the Divisions of Cardiac Surgerya and Cardiology,b Cardio Thoracic
Department, University of Pisa Medical School, Pisa, Italy.
Received for publication Jan 9, 2003; accepted for publication Jan 22, 2003.
Address for reprints: Uberto Bortolotti, MD, Sezione di Cardiochirurgia,
Dipartimento Cardiotoracico, Ospedale Cisanello, Via Paradisa 2, 56124,
Pisa, Italy (E-mail: u.bortolotti@cardchir.med.unipi.it).
J Thorac Cardiovasc Surg 2003;126:1198-9
Copyright © 2003 by The American Association for Thoracic Surgery
0022-5223/2003 $30.00  0
doi:10.1016/S0022-5223(03)00368-4
Figure 1. Gross view of the explanted valve (upper) showing 4
calcified cusps indicated by numbers. Gross view of a segment of
ascending aorta (lower) with thickened and calcified wall.
Brief Communications
1198 The Journal of Thoracic and Cardiovascular Surgery ● October 2003
25 valves with various degrees of fibrosis and calcification. Sur-
gical treatment of postradiation valvular disease has been reported,
predominantly in patients with aortic stenosis.4 The largest surgi-
cal series for radiation-induced valvular heart disease was recently
performed by Handa and associates.7 In this single-center retro-
spective study of 60 patients, they reported a 43% incidence of
isolated aortic valve replacements.
Valvular incompetence is the most frequent hemodynamic ab-
normality in patients with a quadricuspid aortic valve.1-3 The
occurrence of a severe aortic stenosis in the setting of a quadri-
cuspid aortic valve is an exceptional finding and has not been
reported. No such cases were recorded by Turri and colleagues8
among 140 aortic valve specimens retrieved at surgery for aortic
stenosis, and Passik and colleagues9 did not mention any case of
quadricuspid stenotic valve in 646 aortic valves explanted for pure
stenosis. Our patient had severe aortic stenosis and coronary artery
disease approximately 30 years after mediastinal irradiation for a
metastatic seminoma. At operation, a calcified quadricuspid aortic
valve was found characterized by a small accessory cusp between
the right and noncoronary cusps which, according to Hurwitz and
Roberts,1 is the most frequent variety of this malformation.
Quadricuspid aortic valves are prone to develop fibrosis, caus-
ing valve incompetence.3 In the present case, previous mediastinal
irradiation is likely to have contributed to cusp calcification, al-
lowing this malformation to develop an unusual pattern of valve
dysfunction. Concomitant severe calcification of the ascending
aorta and coronary artery disease indicate an extensive cardiac
involvement from the effects of radiation.
References
1. Hurwitz LE, Roberts WC. Quadricuspid semilunar valves. Am J Car-
diol. 1973;31:623-6.
2. Waller BF, Taliercio CP, Dickos DK, Howard J, Adlam JH, Jolly W.
Rare or unusual causes of chronic, isolated, pure aortic regurgitation.
Clin Cardiol. 1990;13:577-81.
3. Bortolotti U, Scioti G, Levantino M, Milano A, Nardi C, Tartarini G.
Aortic valve replacement for quadricuspid aortic valve incompetence.
J Heart Valve Dis. 1998;7:515-7.
4. Veeragandham RS, Golding MD. Surgical management of radiation-
induced heart disease. Ann Thorac Surg. 1998;65:1014-9.
5. Brosius FC, Waller BF, Roberts WC. Analysis of 16 young (aged 15 to
33 years) necropsy patients who received over 3500 rads to the heart.
Am J Med. 1981;70:519-30.
6. Veinot JP, Edwards WD. Pathology of radiation-induced heart disease:
a surgical and autopsy study of 27 cases. Hum Pathol. 1996;27:766-73.
7. Handa N, McGregor CGA, Danielson GK, Daly RC, Dearani JA,
Mullany CJ, et al. Valvular heart operation in patients with previous
mediastinal radiation therapy. Ann Thorac Surg. 2001;71:1880-4.
8. Turri M, Thiene G, Bortolotti U, Milano A, Mazzucco A, Gallucci V.
Surgical pathology of aortic valve disease. A study based on 602
specimens. Eur J Cardiothorac Surg. 1990;4:556-60.
9. Passik CS, Ackerman DM, Pluth JR, Edwards WD. Temporal changes
in the causes of aortic stenosis: a surgical pathologic study of 646 cases.
Mayo Clin Proc. 1987;62:119-23.
Chylous leak after cervical mediastinoscopy
Franc¸oise Le Pimpec Barthes, MD,a Nicola D’Attellis, MD,b Jalal Assouad, MD,a Alain Badia, MD,a
Redha Souilamas, MD,a and Marc Riquet, MD, PhD,a Paris, France
Chylous leak after cervical mediastinoscopy is a rarecomplication.1 To our knowledge, 3 cases have beenreported in the literature.2-4 Three consecutive casesobserved at our institution drew our attention to its
possibly underrated occurrence.
Clinical Summary
PATIENT 1. A 16-year-old girl reported having weight loss and
asthenia after an episode of erythema nodosum. On physical ex-
amination, there was no lymphadenopathy or hepatomegaly. The
chest radiograph demonstrated bulky paratracheal lymph nodes.
Tuberculosis was suspected but could not be confirmed. Diagnosis
was assessed by cervical mediastinoscopy, so as to rule out a
lymphoma. During the procedure, active suction drainage of the
mediastinal bed was used, yielding 300 mL of a milky fluid on
postoperative day 1. A chylous leak was confirmed by the presence
of chylomicrons and triglycerides (14.6 mmol/L) in the fluid.
Suction was discontinued, and a medium-chain triglyceride (MCT)
diet was started. Lymphangiography demonstrated backflow of the
contrast medium from the thoracic duct into the paratracheal
lymph nodes (Figure 1). The chylous leak stopped, the mediasti-
num remained unchanged on successive chest radiographs, and the
drain was removed on the fourth postoperative day.
PATIENT 2. A 56-year-old man had a superior vena caval ob-
struction syndrome. On physical examination, there was no lymph-
adenopathy or hepatomegaly. Chest tomodensitometry demon-
strated a huge right lung tumor and large paratracheal lymph
nodes. Fibrobronchoscopy failed to provide a diagnosis. A medi-
astinoscopy was performed. The mediastinal bed was not drained.
The diagnosis of squamous cell carcinoma was confirmed. On
postoperative day 3, the mediastinoscopy incision leaked a milky
From Service de Chirurgie Thoraciquea and Service d’Anesthe´sie Re´ani-
mation,b Hoˆpital Europe´en Georges Pompidou, Paris, France.
Received for publication Aug 29, 2002; accepted for publication Oct 22,
2002.
Address for reprints: Marc Riquet, MD, PhD, Service de Chirurgie Thora-
cique, Hoˆpital Europe´en Georges Pompidou, 20-40 rue Leblanc, 75015
Paris, France (E-mail: marc.riquet@hop.egp.ap-hop-paris.fr).
J Thorac Cardiovasc Surg 2003;126:1199-200
Copyright © 2003 by The American Association for Thoracic Surgery
0022-5223/2003 $30.00  0
doi:10.1016/S0022-5223(03)00799-2
Brief Communications
The Journal of Thoracic and Cardiovascular Surgery ● Volume 126, Number 4 1199


Severe quadricuspid aortic valve stenosis after mediastinal irradiation 

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Severe quadricuspid aortic valve stenosis after mediastinal irradiation

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