1) Shone complex is a rare congenital heart disease consisting of four left-sided obstructive lesions: coarctation of the aorta, parachute mitral valve, supravalvular mitral ring, and subaortic stenosis. 2) A 33-year-old woman presented with worsening dyspnea and was found to have Shone complex, consisting of previous repair of coarctation, parachute mitral valve, and subaortic stenosis. 3) She underwent successful mitral valve replacement, resection of the subaortic membrane, and left ventricular myectomy, with excellent postoperative recovery.

1. Late Shone complex: A case report and literature review
Jaffar S. Shehatha a, b
, Abdulsalam Yaseen Taha c, *
, Aram J. Mirza d
a
Faruk Medical City, Sulaimaniyah, Region of Kurdistan, Iraq
b
Curtin University, Western Australia, Australia
c
Department of Thoracic and Cardiovascular Surgery, Sulaimaniyah Teaching Hospital and School of Medicine, Faculty of Medical
Sciences, University of Sulaimaniyah, Sulaimaniyah, Iraq
d
Department of Cardiology, Slemani Cardiac Hospital, Sulaimaniyah, Region of Kurdistan, Iraq
a r t i c l e i n f o
Article history:
Received 28 January 2018
Received in revised form 10 March 2018
Accepted 14 March 2018
Available online xxx
Keywords:
Shone complex
Coarctation of aorta
Parachute mitral valve
Supravalvular ring of left atrium
Subaortic stenosis
a b s t r a c t
Shone complex is a very rare congenital heart disease made of coarctation of aorta,
parachute mitral valve, supravalvular mitral ring and subaortic stenosis. Since its first
description by Shone et al., in 1963, literature comes from isolated case reports mainly in
children. Herein, a 33-year old lady with Shone complex is presented. She had had repair
of coarctation of aorta at the age of 5 and mitral valve replacement, resection of subaortic
membrane and left venricular myectomy at the age of 33. Surgery of Shone complex is
expected to yield good outcome if undertaken before the onset of pulmonary
hypertension.
© 2018 The Egyptian Society of Cardio-thoracic Surgery. Publishing services by Elsevier B.V.
This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/
licenses/by-nc-nd/4.0/).
1. Introduction
Shone complex is a very rare congenital heart disease (CHD) described for the first time by Dr. John D. Shone and his
colleagues in 1963 [1]. The complex or syndrome consists of 4 congenital obstructive or potentially-obstructive left-sided
lesions (Fig.1), namely coarctation of aorta, parachute mitral valve (PMV), supravalvular mitral ring and subaortic stenosis [2].
Most cases are diagnosed at childhood. However, late presentation has also been reported [2,3]. Classical complete or
incomplete forms of the syndrome may exist; hence, there is a variation in presentation and outcome [2,3]. Early surgical
intervention before the onset of pulmonary arterial hypertension (PAH) is advised [4,5]. Herein, an adult Iraqi lady with Shone
complex is reported. Despite a history of repair of aortic coarctation at the age of 5, definite diagnosis of the syndrome wasn't
made till the age of 33. The patient underwent a successful open heart surgery with excellent outcome. The case is presented
and the relevant literature is reviewed.
2. Case Presentation
A lady of 33 from Sulaimaniyah, Iraq was referred to the Department of Cardiac Surgery at Faruk Medical City (FMC) on 1st
July 2014 because of worsening effort dyspnea during pregnancy. The patient was evaluated by her general practitioner who
* Corresponding author. Postal Address: Mamostayan Street 112, Road 33, House 5, Sulaymaniyah, 46001, P O Box 1155/64, Iraq.
E-mail addresses: jaffarshehatha@hotmail.com (J.S. Shehatha), abdulsalam.taha@univsul.edu.iq (A.Y. Taha), aram@arammirza.com (A.J. Mirza).
Peer review under responsibility of The Egyptian Society of Cardio-thoracic Surgery.
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Journal of the Egyptian Society of Cardio-Thoracic Surgery xxx (2018) 1e3
Please cite this article in press as: Shehatha JS, et al., Late Shone complex: A case report and literature review, Journal of the
Egyptian Society of Cardio-Thoracic Surgery (2018), https://doi.org/10.1016/j.jescts.2018.03.007

2. discovered a cardiac murmur. Her past medical history was significant for bronchial asthma, transient ischemic attack and
hypothyroidism. Moreover, she had left thoracotomy for repair of coarctation of aorta done at the age of 5. She used to smoke
1e2 cigarettes per day. Her drug history included thyroxine, furosemide, slow-released potassium and aspirin tablets.
Transthoracic echocardiography (TTE) revealed no residual narrowing of the repaired aorta. However, the mitral valve
(MV) was of typical parachute appearance with a mild stenosis yielding a trans-valvular pressure gradient of 28 mm Hg
during exercise. The aortic valve (AV) was of normal tri-leaflet structure but there was a mild to moderate central stenosis due
to a sub-aortic membrane (SAM) 1 cm beneath the AV. The left ventricular size and function were normal with an ejection
Fig. 1. An illustration of Shone Complex showing the 4 defects with the direction of blood flow.
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3. fraction (EF) of 63%. These TTE findings coupled with history of a previously-repaired aortic coarctation favoured a diagnosis
of Shone complex.
At the time of operation, the ascending aorta, pulmonary artery and pulmonary venous drainage were all normal. The left
atrium (LA) was significantly enlarged. The MV was bi-leaflet. Both commissures were fused together causing a significant
stenosis. The papillary muscles were big in size reaching the edge of the leaflets but there was no supra-valvular ring. In
addition, there was a very small SAM (1 mm  2 mm) in the region of the left ventricular outflow tract. The MV was replaced
by a prosthetic valve (St Jude 27 mm). Left ventricular myectomy was performed along resection of the SAM. The post-
operative course was smooth. She stayed in the intensive care unit (ICU) for 1 day and was discharged home on the 7th
postoperative day. Warfarin was prescribed to achieve a target international normalized ratio (INR) of (2.5e3.5). The patient
was seen 6 weeks after operation and found to have no more shortness of breath. Postoperative chest radiograph revealed
normal-sized heart and clear well-expanded lungs.
3. Comments
Shone's complex is a rare congenital anomaly; by 2007, fewer than 100 patients have been reported in the literature
[Subramanyan cited in 5]. Since its first description by Shone et al., most of the literature comes from isolated reports [2].
The deformity of PMV refers to a bi-leaflet MV with 2 commissures in which the chordae converge to insert into one major
papillary muscle instead of two [2]. The supravalvular ring of LA is a circumferential ridge of connective tissue that arises at
the base of the atrial surfaces of the mitral leaflets and protrudes into the MV inlet. Two types of subaortic stenosis were
originally described (muscular and membranous). The aforementioned combination represents the classical complex.
However, incomplete forms with two or three lesions are also described [2,5]. The case reported herein is considered
incomplete as it lacked a supra-valvular mitral ring. St. Louis et al. had only 2 cases of complete Shone complex out of 28 cases
(7.1%) [4]. In contrast, Bolling et al. [cited in 3] described a complete form of the syndrome in 19/28 (68%) of their patients.
The diagnosis of Shone complex is essentially based on clinical grounds and must be confirmed by echocardiography and/
or magnetic resonance imaging (MRI) [2]. Although most cases are detected at childhood [5], late diagnosis at adulthood has
been occasionally reported [2,3]. In the study of St. Louis et al., 28 patients with Shone complex were diagnosed at a median
age of 14 days with 46% of them presenting within the first week of life [4]. Robich et al. reported 2 adult patients aged 22 and
25 years with this complex [5]. Goswami et al. [cited in 5] reported Shone's anomaly in a young gravid female mimicking
preeclampsia at 25 weeks gestation. It is extremely unusual for a patient to remain largely asymptomatic throughout
childhood and get incidentally detected during adulthood [5]. The present case was actually diagnosed as coarctation of aorta
at the age of 5 while PMV and aortic stenosis were discovered 28 years later; thus the diagnosis of Shone complex was late.
The formulation of an appropriate operative strategy for Shone complex remains problematic. This is due to variability of
presentation and severity of individual lesions [4]. Operative strategies for this complex group should be individualized [4].
Outcomes have improved due to better diagnostic imaging, care, and surgical management [3]. Most reported patients
required multiple operations [3]. In one study, 17/27 patients (63%) underwent 2 operations [4]. In another study, 84 oper-
ations were done for 28 patients over 23 years with an average of 3 operations per patient [Bolling et al. cited in 3]. Early
aggressive surgical repair of all defects of Shone complex in one session has historically yielded poor results [4]. Initial
conservative approach to MV deformities has been recently shown to improve operative survival [4]. Mitral valve repair along
with resection of supra-mitral ring is preferable over valve replacement [5].
The complexity of surgery of Shone syndrome is well portrayed by the 2 patients reported by Robich et al. The first was a
man of 25 who underwent 5 median sternotomies while the second was a lady of 22 and underwent 4 operations. Worthy to
note that despite severe PAH and high pulmonary vascular resistance (PVR), both patients responded to conventional valve
surgery that rapidly reversed PAH and PVR [3].
In regard to prognosis, studies have shown that severity of MV obstruction is a major predictor of poor long-term survival
[2,3]. Moreover, patients who develop PAH have more dismal prognosis [3]. A good outcome is possible in patients with
Shone's complex provided the surgical intervention is undertaken early before the onset of PAH [5].
In a summary, this is a very rare case of incomplete Shone complex in an adult. Although the exact diagnosis wasn't made
at childhood, early repair of aortic coarctation could have contributed to her minimal symptoms. The patient was successfully
managed by conventional valve surgery.
References
[1] Shone JD, Sellers RD, Anderson RC, Adams Jr P, Lillehei CW, Edwards JE. The developmental complex of “parachute mitral valve,” supravalvular ring of
left atrium, subaortic stenosis, and coarctation of aorta. Am J Cardiol 1963;11:714e25.
[2] Lorenzo N, Claver E, Ali J, Aguilar R. Incomplete Shone's complex in the sixth decade of life: echo and cardiac magnetic resonance imaging assessment.
Europ Heart J Adv Access publ 2016. https://doi.org/10.1093/eurheartj/ehw390.
[3] Robich MP, Stewart RD, Zahka KG, Krasuski RA, Hanna M, Blackstone EH, et al. Two cases of late Shone syndrome with pulmonary hypertension:
heartelung transplant or valve surgery? World J Pediatr Congenital Heart Surg 2016;7(1):100e3. https://doi.org/10.1177/2150135115577671.
[4] St Louis JD, Bannan MM, Lutin WA, Wiles HB. Surgical strategies and outcomes in patients with Shone complex: a retrospective review. Ann Thorac Surg
2007;84:1357e63.
[5] Narvencar Kedareshwar PS, Jaques e Costa Ana Karina, Patil Vijaysinh R. Shone's complex. JAPI 2009;57:415e7. Available from: www.japi.org/may_
2009/article_12.pdf?q¼shone.
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Please cite this article in press as: Shehatha JS, et al., Late Shone complex: A case report and literature review, Journal of the
Egyptian Society of Cardio-Thoracic Surgery (2018), https://doi.org/10.1016/j.jescts.2018.03.007