Discussion

Congenital left ventricular outpouchings have long been classified based on the following three characteristics as narrow connecting neck, synchronous contraction of protrusion, and presence of all three layers – myocardium, endocardium, and pericardium – in the outpouching.Reference Zhang, Shen, Wu, Xue and Zhang ⁴ However, a review published in 2014 provided a new classification, where congenital left ventricular outpouchings were classified according to left ventricular elliptical shape preservation, wall thickness, and regional wall motion.Reference Malakan Rad, Awad and Hijazi ⁵ According to the new classification, our patient was classified as left ventricular outpouching type 1, because the left ventricle could not retain its elliptical shape, and the wall thickness did not decrease.

Ohlow et al investigated a large series of patients from prenatal age to adulthood and identified 664 patients with left ventricular aneurysm or left ventricular diverticulum since the first description of the condition in 1816. Today, around 800 cases have been published.Reference Ohlow, Brunelli and Lauer ^{6 ,} Reference Ozaki, Sato, Inoue and Wakita ⁷ However, multiple ventricular diverticula are very rare.Reference Alkan, Bilgin, Zihni and Nalbantgil ^{1 ,} Reference Hubacek, Brydie and Jackson ^{8 ,} Reference Tandar, Meredith and Anderson ⁹ Congenital left ventricular outpouchings should also be distinguished from other causes of left ventricular aneurysm such as post-cardiac surgery, trauma, Chagas disease, tuberculosis, and idiopathic.Reference Nunes, Kreuser and Ribeiro ^{10 ,} Reference Wei, Hui, Sheng-Yu and Xin ¹¹

Although it is a rare cause of non-ischaemic cardiomyopathy, the finding of apical wall dyskinesia or apical ventricular aneurysm in a patient with symptoms of heart failure and no evidence of myocardial ischemia should prompt the consideration for Chagas disease. The left ventricular aneurysms due to Chagas disease are very difficult to distinguish from congenital left ventricular aneurysms without the knowledge of past history and the serology. Therefore, when apical aneurysm is seen, Chagas disease needs to be ruled out by doing serological tests. In addition, travel to places where the disease is common needs to be questioned.Reference Andrade, Marin Neto and Paola ¹²

Although the exact aetiology of the left ventricular diverticula remains unclear, an embryological developmental defect has been proposed. An outpouching can occur due to maldevelopment of myocardial intra-trabecular sinusoids in the first 2–3 weeks of embryonic life.Reference Papagiannis, Van Praagh and Schwint ¹³ Most left ventricular diverticula are located at the left ventricular apex.Reference Ozaki, Sato, Inoue and Wakita ⁷ When left ventricular diverticulum is associated with midline thoracoabdominal congenital abnormalities, diaphragmatic and sternal defects, and partial absence of the inferoapical pericardium, the condition is called Cantrell syndrome.Reference Makkuni, Kotler and Figueredo ¹⁴

The natural history of left ventricular diverticulum has not been studied systematically. The age of diagnosis may range from the prenatal period to the eighth decade. Interestingly, the size of the protrusion might increase, decrease, or remain unchanged with time. If Cantrell syndrome is not accompanied by any other condition, it may remain silent and go unnoticed for a long time. However, several complications have been reported in association with ventricular diverticulum, including cardiac rupture, embolic events, congestive heart failure, endocarditis, ventricular arrhythmias, and valvular abnormalities. In our case, a patient who had no complaints for up to 2 years presented with increasing effort dyspnoea. Echocardiography revealed mild impairment of left ventricular systolic function. A small number of adult cases of heart failure due to ventricular diverticulum have been reported in the literature.Reference Handler and Walker ^{15 ,} Reference Lachaud, Tivane, Menete, Narayanan, Sidi and Ferreira ¹⁶ These cases may be related to the size of the diverticulum or aneurysm. In our case, although the diverticular dimensions were small, they were multiple, and apical settlement may lead to heart failure.

Currently, there is no consensus on treatment of the left ventricular diverticula due to rarity of the condition. Some authors have recommended immediate surgical resection to prevent complications.Reference Parthenakis, Kochiadakis, Patrianakos and Hamilos ¹⁷ However, the decision depends on parameters such as diverticulum size, presumed risk of rupture, occurrence of a thromboembolic incident, or serious ventricular arrhythmia.Reference Marijon, Ou and Fermont ¹⁸ Chesini et al reported a successful surgical intervention in a patient with multiple left ventricular diverticula who presented with multiple embolic episodes. No recurrent embolism was observed during 1 year follow-up. However, surgical treatment could only be performed on one of four diverticula.Reference Chesini, Sangera and Howard ¹⁹ Harris et al reported a symptomatic young lady who presented with multiple diverticula and was followed up with heart failure treatment and anticoagulant therapy; the patient’s symptoms were seen to improve.Reference Harris, Wong and Lantz ²⁰ We treated our patient with β-blockers, spironolactone, ramipril+hydrochlorothiazide, and warfarin. On follow-up, she remains asymptomatic. Therefore, surgical treatment was not considered.