Discussion

Cardiac tumour is rare in infants with a prevalence about 0.05–0.14%, of which only 1–2% comprises vascular tumours.Reference Issacs ^{1 ,} Reference Mackie, Kozakewich, Geva, Perez-Atayde and Mulliken ² Following the successful surgical treatment of infantile cardiac haemangioendothelioma by Ilbawi et al,Reference Ilbawi, DeLeon, Riggs and Hernandez ³ only 37 cases of haemangioma, haemangioendothelioma, or angiosarcoma in the heart of infants have been reported. Supplementary Table S1 summarises the patient characteristics, clinical presentation, surgical procedure, and outcome of these cases.

Echocardiography, either prenatal or postnatal, usually establishes the diagnosis of the cardiac tumours accurately.Reference Issacs ¹ The typical echographic feature of a cardiac vascular tumour is mixed echogenicity with echogenic and hypoechogenic parts. Blood flow signal is absent by colour flow mapping in most patients, as the abnormal vascular structures are microscopic; nevertheless, the main feeding artery could be demonstrated occasionally.Reference Mackie, Kozakewich, Geva, Perez-Atayde and Mulliken ² Unlike the more common rhombdomyoma or fibroma, cardiac vascular tumour is secretory in nature, and pericardial effusion is frequently presented.Reference Issacs ^{1 ,} Reference Sharma, Hirata and Mosca ⁴ In the reported 37 cases, 27 of them (73.0%) presented with pericardial effusion. Cardiac tumour or pericardial effusion was detected by foetal echocardiography in 18 patients in this review (48.6%), of which 14 (77.8%) were found to have pericardial effusion prenatally; in utero pericardiocentesis was performed on one patient at 20th week of gestation owing to severe hydrops.Reference Sharma, Hirata and Mosca ⁴ Congenital thyroid dysfunction, diaphragmatic hernia/eventration, and infection are other common causes of foetal pericardial effusion in the absence of hydrops.Reference Cartagena, Levin, Issenberg and Goldman ⁵ The presence of cardiac mass together with pericardial effusion should raise the suspicion of the diagnosis of vascular tumour. Teratoma is the other tumour frequently related to pericardial effusion and most of them originate from the pericardium, whereas cardiac vascular tumours usually arise from the heart itself.Reference Issacs ^{1 ,} Reference Cartagena, Levin, Issenberg and Goldman ⁵

In this review, six infants (16.2%) with cardiac vascular tumour were asymptomatic and the diagnoses were made incidentally by prenatal or postnatal echocardiography. Cardiac tamponade and cardiopulmonary distress were the usual presentations in symptomatic patients. The tumour could occur at any place of the heart, with the majority at the right atrium (27 of 37, 73.0%). Of the patients, three (8.1%) had tumour associated with cutaneous haemangiomas, and one (2.7%) had multiple visceral vascular malformations, resulting in fatal pulmonary haemorrhage. Foetal arrhythmia or cardiopulmonary compromise had occasionally developed, which resulted in preterm delivery in four foetuses (10.8%). In the presented case, we delivered the boy twins at 34th gestation week soon after the diagnosis, even without significant foetal cardiopulmonary compromise, and performed prompt surgical intervention in his very early life, concerning that cardiac tamponade might develop and the risk of respiratory distress syndrome or other neonatal morbidities was already low at this gestational age.

Surgical resection remains the choice of therapy for infants diagnosed with cardiac vascular tumour, especially for those with symptoms. Of the reviewed cases, 28 patients (75.7%) were treated with surgical resection, and complete excision was achieved in 16 patients (43.2%). No tumour recurrence was reported after complete excision. The close relationship of the tumour with the conduction system and coronary arteries might preclude the complete excision; however, tumour regression or even complete resolution might be anticipated after incomplete excision or conservative treatment.Reference Mackie, Kozakewich, Geva, Perez-Atayde and Mulliken ^{2 ,} Reference Minsart, Ridremont and Lecocq ⁶ Even for those more aggressive haemangioendotheliomas and malignant angiosarcomas of the heart, scarce literature also showed satisfied surgical outcome.Reference Beaton, Kuttler and Hassan ^{7 ,} Reference Rosenkranz and Murphy ⁸ In those with cardiac tamponade but unresectable tumours, drainage alone with or without tumour biopsy is advised. Despite with unknown mechanism, systemic steroid therapy was applied in the management of infantile cardiac vascular tumours in several reports and carried favourable outcomes.Reference Mackie, Kozakewich, Geva, Perez-Atayde and Mulliken ^{2 ,} Reference Dresse, David and Hume ^{9 ,} Reference Chang, Young, Chuu and Lue ¹⁰ Its use, however, needs further investigation and should be reserved for those who are not candidates for surgical resection or with significant residual tumour after incomplete excision.

In this article, we report one small premature patient with cardiac haemangioma treated by complete excision in the neonatal period and give a review of infantile cardiac vascular tumour. Infantile cardiac vascular tumours are most commonly located in the right atrium and are frequently associated with pericardial effusion. The diagnosis can often be made by foetal echocardiography and the foetus should be delivered promptly once foetal cardiopulmonary compromise develops. Surgical removal of the tumour provides adequate result and is advised in most patients, even for those premature neonates such as the presented case. For foetuses with cardiac tumour and pericardial effusion, one algorithm regarding the decision making from prenatal diagnosis and timing of delivery to postnatal management is proposed (Fig 2).

Figure 2 Algorithm of decision making for foetus prenatally diagnosed with cardiac tumour and pericardial effusion. OBS=observation.