Research Article
Sinus of Valsalva Aneurysm – A Possible Association with Infection of Lymphatic Mode of Spread: Report of 15 Cases
Lupinski Ryszard1,2*
1Department of Cardiothoracic Surgery, National Heart Department of Centre, Singapore
2Department of Cardiothoracic Surgery, University of Cape Town, South Africa
*Corresponding author: Lupinski Ryszard, Department of Cardiothoracic Surgery, National Heart Department of Centre, Singapore
Published: 13 Jul, 2018
Cite this article as: Ryszard L. Sinus of Valsalva Aneurysm
– A Possible Association with Infection
of Lymphatic Mode of Spread: Report of
15 Cases. Clin Surg. 2018; 3: 2013.
Abstract
Objective: Sinus of Valsalva Aneurysm (SoVA) is a rare cardiac defect. Both congenital and
acquired aetiology of SoVA have been proposed. It was the aim of this study to describe the clinical
presentation of SoVA and to investigate the possible association with infectious disease in these
patients.
Methods: Fifteen patients with SoVA were identified. Patient case notes were retrieved and reviewed.
Patients were 7 females and 8 males with a mean age of 31 years (range 10-56). Seven patients were
South African Bantu, six were Chinese and two were Caucasians.
Results: Chronic chest infections not responding to routine antibiotics treatment were diagnosed
in seven patients, tuberculosis and syphilis were each diagnosed in two patients and one patient
had pharymgitis. Three patients had no significant medical history of infection. Intrathoracic
lymphadenopathy was observed in 10 of 13 patients with recorded findings.
Conclusion: Sinus of Valsalva Aneurysm seems to have strong association with infections of
lymphatic mode of spread. These infections have developed prior diagnosis of SoVA was made in
majority of patients.
Introduction
Sinus of Valsalva Aneurysm (SoVA) is a rare cardiac defect. The first report of SoVA is attributed to Hope who reported a case of ruptured SoVA in 1839 [1]. A comprehensive review by Nowicki in 1977 identified only 175 reported cases [2]. Unruptured SoVA is usually asymptomatic although they can produce aortic valve incompetence, coronary artery compression or fistulae, conduction disturbances or pulmonary outflow obstruction due to compression of adjacent structures [3]. Rupture of SoVA is usually intracardiac and leads to development of congestive cardiac failure and arrhythmias. Both congenital and acquired aetiology of SoVA have been proposed. Deficiency of elastic tissue at the site of congenital SoVA has been histologically demonstrated [3]. Acquired aneurysms have been described secondary to arteriosclerosis, syphilis, infective endocarditis, cystic median necrosis and trauma [3].
Objective
It was the aim of this study to describe the clinical presentation of SoVA and to investigate the possible association with infectious disease in these patients.
Patients and Method
15 patients with Sinus of Valsva Aneurysm were identified. Nine patients were treated at Groote
Schuur Hospital and the Red Cross War Memorial Children’ Hospital, (Cape Town, South Africa)
and six at National Heart Centre (Singapore, Figure 1).
Patient case notes were retrieved and reviewed. Patient demographic features (gender, race,
age, occupation and region), clinical presentation including evidence of infectious disease, results
of investigations (chest X-Rays, blood tests) and surgical or autopsy findings were studied. Syphilis
was diagnosed where TPHA and VDRL tests were both positive. Tuberculous lymphadenitis was
diagnosed from tissue samples. Lymphadenopathy was detected by means of clinical appearance
(palpation, hoarseness), chest X-ray criteria (stretching or compression of the trachea, unilateral
elevation of the diaphragm, atelectasis, [4] Figure 2). Cardiac lesions were diagnosed on
echocardiography and/or angiography.
Table 1
Table 2
Results
Demographics
Between 1967 and 1996, twelve cases of Sinus of Valsalva were
identified. Patients were four females and 8 males with a mean age
of 26.9 years (range 10-48). 4 patients were of Negroid race, 5 were
Caucasians and 3 were Coloured. All patients were coming from high
TB prevalence areas (Table 1).
Clinical presentation
Clinical findings are summarised in Table 2. Two patients clinical
data (9,12 Table 2) were incomplete. Mean length of history of
presentation was 21.8 months (range 3-60) at operation. Two patients
had tuberculous lymphadenitis and TB could be suspected in another
8 patients on the basis of reported clinical findings. Two of the patients
had diagnosed syphilis. Intrathoracic lymphadenopathy was observed
in 8 patients, two of whom also had cervical lymphadenopathy. Two
had no record of lymphadenopathy in patient case notes, and in
another two patients no lymphadenopathy was observed.
Sinus of Valsalva Aneurysm was associated with aortic valve
incompetence in 8 patients, with 4 patients also having mitral valve
incompetence. One patient had also coarctation of the aorta. Ten
patients had signs and symptoms of congestive cardiac failure. ECG
demonstrated conduction delay abnormalities in ten patients and six
of the patients had Q waves on ECG despite unobstructed coronary
flow on angiography (T Figure 3).
Surgical findings
Eleven patients underwent surgical repair of aneurysm. In three
cases „windsock” form of aneurysm was reported among SoVA
patients (Figure 1). One of the patients had aneurysm left untouched
(patient 9) due to high risk of RCA damage during closure of the
RCA-RA cameral fistula. Three patients had aortic valve replacement.
Four patients also had repair of associated Annular Subvalvular Left
Ventricular Aneurysm (ASLVA). The Sinus of Valsalva Aneurysm
was cited in the non coronary sinus in 8 cases, in the right coronary
sinus in 6 cases and in the left coronary sinus in 4 cases. In 6 cases
there were multiple aneurysms. In one case it was unruptured
aneurysm of "windsock” type communicating with right atrium via
cameral RCA-RA fistula. Fistulae communicating between Sinus
of Valsalva Aneurysm and the heart chambers were observed in all
cases, with multiple fistulae in three patients. In 6 cases this was to the
left ventricle, and in 7 cases to the right atrium, and in 2 cases to the
right ventricle.
Histological findings
Aneurysmal wall was taken for histopathology evaluation in
five and aortic valve in two SoVA cases. Specimens from left atrial
appendage and right atrial appendage were each taken in one case.
Three patients had no tissue taken for histopathology. The wall of
Sinus of Valsalva Aneurysm was composed of collagenous connective
tissue lined by endothelium in five specimens. Hyalinisation was the
finding in two SoVA wall specimens. Inflammatory infiltrate was
observed in three and calcification was observed in two specimens.
Besides features mentioned above, two of the wall specimens were
consisted of three basic vessel’s layers, namely intima - endothelium,
media - muscle fibers and adventitia - epithelium (Figure 2). The
pathological changes mostly affect the media layer, where the muscle
fibres were replaced by fibrous tissue. Tissue of left atrial appendage
showed vessels endothelial thickning and fibrosis in one case, and in
one case vessels' thickening was observed within tissue of right atrial
appendage. Aortic valve was fibrosed in two cases and hyalinisation
was observed in one.
Complications of surgery:
• Conduction disturbances – 14
• Q waves/ST abn – 12
• Chylopericardium – 1
• Chylothorax – 1
Figure 1
Figure 2
Discussion
The first description of aneurysmal enlargement of Sinus of
Valsalva with intracardiac rupture has been attributed to Hope
(1839). Thurnam (1840) published a series of six cases. Abbot in 1919
proposed and Edwards in 1957 established concept of congenital
SoVA, who histologically demonstrated the deficiency of elastic
tissue at the sight of congenital SoVA [1]. A comprehensive review by
Nowicki in 1977 only identified 175 reported cases [2].
The high incidence of infections, developed prior diagnosis
of SoVA was made, in patients from this study with the associated
lymphadenopathy supports an infective origin of SoVA and lymphatic
spread as the mode of spread of these infections. Syphilis as a cause
of SoVA was initially emphasised by Smith in 1914 [3], reported by
others [1,5,11], it has been diagnosed in two patients from this study.
Tuberculous lymphadenitis has been diagnosed in two patients from
this study, finding not reported in the literature previously. Sometimes
infective endocarditis can associate with SoVA [6,7]. Mediastinal
lymphadenopathy seems to play important role in the pathology of
SoVA. It has been diagnosed in ten of thirteen patients with recorded
finding, including all four patients with associated Bantu aneurysm.
The similarity in the aetiology of Sinus of Valsalva Aneurysm and
Bantu Aneurysm was first noted by Chesler [8]. Association of SoVA
and Bantu Aneurysm was observed in four cases from this study and
has been reported by others [9,10]. The similar morphology of some
SoVA and Bantu Aneurysm, „finger-like” or „windsock” type of
morphology (Figure 3), and the similar clinical presentation of these
patients' further supports Chesler’s concept of the same aetiology of
these two rare cardiac lesions. Lymphatic spread of infections with
associated post inflammatory destruction of mediastinal lymph nodes
in both - SoVA and Bantu aneurysm, seems to play significant role in
the development of these rare cardiac aneurysms.
Conclusion
• Sinus of Valsalva Aneurysm has strong association with
infections of lymphatic mode of spread.
• Sinus of Valsalva Aneurysm can associate with Bantu
Aneurysm and both may have similar infective ethiology.
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