Cardiovascular Journal of Africa: Vol 30 No 3 (May/June 2019)

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CARDIOVASCULAR JOURNAL OF AFRICA • Volume 30, No 3, May/June 2019 AFRICA e3
Case Report
Second recurrence of familial atrial myxomas:
mother and daughter simultaneously
Zehra Bayramoglu, Kerem Oral, Mehmet Ezelsoy, Belhhan Akpinar
Abstract
Sporadic cardiac myxomas rarely recur, however recurrence
rates are higher in patients with a familial aggregation or
Carney complex. Carney complex is characterised by multi-
ple mucocutaneous lesions and accounts for up to two-thirds
of familial cardiac myxomas. A second recurrence is very
rare, even in the case of Carney complex. We report on two
cases of recurrent cardiac myxoma, a mother and daugh-
ter, who concurrently presented with a second recurrence
of atrial myxomas. The time interval between the first and
second recurrence following surgery was four years in both.
The possibility of repeat recurrence of cardiac myxomas
demonstrates the importance of regular echocardiography to
detect recurrence and to prevent the potential complications
associated with cardiac myxomas. Family screening should be
recommended for familial myxomas.
Keywords: cardiac myxoma, Carney complex, familial cardiac
myxoma, atrial myxoma
Submitted 12/12/17, accepted 22/1/19
Published online 6/2/19
Cardiovasc J Afr 2019; 30: e3–e6
DOI: 10.5830/CVJA-2019-008
Myxomas that are mesenchymal tumours are typically sporadic
and isolated. This disease occurs as a familial form in only
around 7% of all cases. This group of patients is younger
and they frequently have multifocal tumours; some suffer
from additional neoplasms called complex myxoma or Carney
complex.
It is important to manage cardiac tumours in patients with
Carney complex because the most common cause of death in
these patients is heart-related problems; 57% of all-cause deaths
include cardiac myxoma, emboli, heart surgery complications
and cardiac arrhythmia.1 Treatment is surgical excision and
should be performed on detection due to embolic complications.
The relapse risk for sporadically occuring myxomas varies
between one and 3% and it is increased significantly, at a rate
of 10–21%, for patients with familial aggregation or Carney
complex.2 We present two cases, a mother and daughter, with
a second recurrence of atrial myxomas within four years of
surgical resection.
Case report
A 23-year-old daughter and 50-year-old mother presented in
November 2016 with fatigue and exertional dyspnoea. The
family history revealed that both women had a history of left
atrial myxomas and two concurrent cardiac surgeries that were
four years apart. Transthoracic echocardiography (TTE) in 2016
indicated the second recurrent myxomas in the left atrium and
they were both referred to our hospital.
The mother and daughter had similiar symptoms. Both
patients revealed tachycardia with a regular heart rate at about
115 beats per minute, pedal oedema and hepatomegaly.They had
hyperpigmentation and nevi on their bodies, similar to Carney
syndrome.
Case 1: The daughter had a history of cardiac operations for
left atrial myxoma in 2008 and 2012. The history of the young
woman revealed that she was in the first trimester of pregnancy
at the first reccurrence and she had a second cardiac operation.
At the time of the second recurrence she was 16 weeks’ pregnant.
TTE of the young woman revealed a stable mass (40 ×
25 mm) near the left superior pulmonary vein orifice and a
pediculated mass (60 × 45 mm) in the left atrium, which was
attached to the septum foramen ovale. The tumour prolapsed
from the left atrium into the left ventricle through the mitral
valve orifice, causing mitral valve obstruction and moderate
regurgitation (Fig. 1).
Following the decision for surgery for the cardiac mass, a
medical abortion was performed after getting ethical and legal
approval from her family. Five days later, she had a third cardiac
operation with a minimal invasive technique (port access).
Surgical excision of the intra-cardiac myxoma was performed
through a right anterolateral mini-thoracotomy under the breast.
Cardiopulmonary bypass was performed via the internal jugular
vein, femoral vein and femoral arterial cannulation.
The patient was cooled to 26°C and an incision was made
in the left atrium with a ventricular fibrillation technique. A
large jelly-like left atrial mass (60 × 45 mm) was seen with
Department of Cardiovascular Surgery, Istanbul Bilim
University, Florence Nightingale Hospital, Istanbul, Turkey
Zehra Bayramoglu, MD,
Mehmet Ezelsoy, MD
Department of Cardiovascular Surgery, Florence
Nightingale Hospital, Istanbul, Turkey
Kerem Oral, MD
Belhhan Akpınar, MD