Primary cardiac tumors in newborns

Yulia Pisklova1, Igor Polivenok1, Daria Kulikova1, Yaroslav Shafer1, Olga Buchneva1, Valery
Bojko1, William Novick2
1
Zaycev V.T. Institute of General and Urgent Surgery, Kharkiv, Ukraine
2
Novick Cardiac Alliance, Memphis, USA
Introduction: Primary cardiac tumors are quite rare pathology in children, especially in
newborns. The frequency of this disease in the general population is about 0.14%, decreasing to
0.0017 - 0.028% among live-born children, according to reports and postmortem studies. (2, 3,
5) In pediatric population the most common primary cardiac tumor is rhabdomyoma(40-60% ),
then followed teratoma (15-19%), fibroma (17-19% ) and others. (5, 6) Myxoma in children
occurs with a frequency of 2-10 % among all cardiac tumors. (5, 7) In contrast in adults it occurs
in 50-90 % of cases of histologically verified cardiac tumors(4, 7, 8). Most often myxomas are
localized in the left atrium (75-90%), the least in the right atrium (7-20%), localization in the
ventricles of the heart is rare - 4-10%. (4, 8)

Primary cardiac tumors are detected in about 0.8-1.3% of the total number of operated patients in
the cardiac clinics. The accumulation of clinical experience, as well as the intensive development
of non-invasive diagnostic technologies has led to an increased number of reported cases.

A timely and accurate diagnosis is very important for successful treatment of cardiac tumors and
can provide a prognosis for the neoplastic process.

One of the main problems in the early diagnosis of cardiac tumors is the lack of specific
symptoms in this group of patients. Clinical manifestations of significant neoplastic process in
the fetus may be arrhythmia, heart failure, edema and polyhydramnios. Cyanosis, respiratory
failure, myocardial dysfunction, signs of outflow and inflow tract obstruction, as well as valve
embolization, may appear in the neonatal period. (1, 5, 6) Also cardiac tumor may cause the
sudden death. (1, 5, 9) Identification of the tumor could be possible in the prenatal period by
routine prenatal echocardiography. However, due to low suspicion of physicians and suboptimal
coverage of prenatale chocardiography, detection of cardiac tumors is rare in the fetus. In the
postnatal period, the main diagnostic methods are echocardiography, spiral CT and cardiac MRI.
The gold diagnostic standard is biopsy with followed histological assessment. (1, 5, 6)

Treatment of the cardiac tumors is surgical and should be carried as soon as possible after
diagnosis, because even in the case of benign morphological characteristics, which with the
exception of teratoma, is extremely difficult to reveal before surgery, may result in a
compromised clinical course; obstruction of the valves, conduction system damage,
thromboembolic complications are all possible. (5-7). However, the surgical strategy and
outcome depends on the tumors location and its histological type. (5, 6)

We present two clinical cases of neonatal cardiac tumors with critical hemodynamic disorders
caused by tumor obstruction of the heart valves.

Clinical case 1: Child S., 1 month, male, presented to the cardiac surgery unit in critical
condition with severe cyanosis and episodes of circulatory collapse. The history was
unremarkable, normal delivery after second normal pregnancy in gestational age 40 weeks , birth
weight is 3900g. Two days before admission baby developed profound vomiting, which was
considered as a manifestation of intestinal infection, but the next day the cyanosis appeared and
the child was referred to our cardiac unit. On admission the child's condition was critical, SpO2 -
54%, shock, oliguria. Echocardiographic examination revealed a mass in the right atrium
obstructing tricuspid inflow (Figs. 1 & 2)

Figure 1: Intracardiac tumor 2,31×2,35 cm into the right atrium, right-to-left flow on patent
foramen ovale.

Fig. 2: Tumor obstructs and protrudes into the tricuspid valve.

Emergent surgery was performed utilizing routine bi-caval cannulation for cardio-pulmonary
bypass and a tumor was excised from the attachment to the atrial septum.
Fig. 3: The extracted tumor.

Histological conclusion: myxoma .

The postoperative period was uneventful. Observation at 6 months showed no signs of tumor
recurrence.

Clinical case 2: Child M., 4 days, female, was urgently taken to the hospital for circulatory
collapse and SpO2 falling to 70 %. The child was born 41 weeks of gestation with a weight of
3450g. Maternal past history was complicated with chronic toxoplasmosis and asymptomatic
bacteriuria. Echocardiography upon arrival to our unit revealed a tumor of left ventricular
outflow tract (Figs. 4 & 5).

Fig. 4. Intracardiac tumor almost completely obstructs left ventricle outflow tract.

Fig. 5. Pressure gradient across the LVOT is 67 mm Hg.

Emergent surgery was performed and a tumor 8 mm in diameter, based on the ventricular
septum, was successfully removed under cardiopulmonary bypass.

Fig. 6. Extraction of the tumor through the aortic valve.

Fig. 7. Extracted tumor measurement on OR table.

Histological conclusion: fibromyxoma .

The postoperative period was uneventful. Observation for 5 months showed no signs of tumor
recurrence.

Discussion. Despite the relatively low incidence of cardiac tumors in children, we shouldn’t
forget about the possibility of their development, including newborns. Echocardiography is a key
method of diagnosis of such conditions and should be performed as soon as possible for any
deterioration of the newborn, especially with the appearance of cyanosis or hemodynamic
collapse.

While tumors in general are not urgent conditions, cardiac manifestations of tumors in a child
can carry extremely dramatic. The key here is rapid diagnosis and urgent surgery. Surgical
treatment has been and remains the treatment of choice . The short and long-term prognosis for
patients operated on because of benign cardiac tumors are usually favorable. (10)

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