Background

Infantile hepatic hemangioma (IHH) is a rare proliferative endothelial cell tumor. It appears to be a benign tumor, however, it may lead to poor outcomes because of severe complications such as congestive heart failure (CHF), which occurs in 15% of infants with this disease [1]. More rarely, IHH is concurrent with congenital heart disease (CHD). Herein we present a patient with IHH, patent ductus arteriosus (PDA), and atrial septal defect (ASD). Meanwhile, we review and summarize the injury of IHH on the heart, and related outcomes.

Case presentation

A 10-day-old boy was born at 38 weeks’ gestation and had tachypnea at 65 breaths per minute. The liver margin was palpable 4 cm below the left costal margin. The heart rhythm was normal but a grade II-IV systolic murmur could be heard. Laboratory tests showed that his brain natriuretic peptide (BNP) level was greater than 5000 pg/ml. His CKMB was 110 U/L, C-reactive protein was 23.4 mg/L, alpha fetoprotein was greater than 1210 ng/ml, PTA 54%, INR 1.6, APTT 50 s, FIB 1.5 g/l, D-dimer 1064 μg/L, HGB, 123 g/L, MCH 36.4 pg, total bilirubin (BilT)196.7 umol/L, BilD 13.2 umol/L, ALT 44 U/L, and AST 23 U/L. Blood gas analysis revealed that the carbon dioxide pressure was raised to 53.4 mmHg and the oxygen partial pressure was 62.5 mmHg.

Echocardiography demonstrated a small PDA of 1.5-1.8 mm with right to left shunting, a large ASD with left to right shunting, and severe right atrial and right ventricular enlargement. A severe systolic pressure gradient of 70 mmHg suggested marked elevation of pulmonary artery pressure at the near systemic level. (Fig. 1) Color Doppler scanning of the liver displayed abundant blood flow in the lesion. (Fig. 2) The left hepatic vein was dilated to 8 mm with two great branches feeding the mass. (Fig. 3)The right hepatic artery and a branch arising from the abdominal aorta were also in close association with the lesion.

Fig. 1
figure 1

IHH (Infantile hepatic hemangiomas) on abdominal ultrasound, in the left hepatic lobe. CDFI(Color doppler flow imaging) can be detected with abundant blood flow signals

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Fig. 2
figure 2

Views of the IHH from contrast-enhanced CT (Contrast-enhanced computed tomography). The left hepatic lobe is irregularly enhanced with a slightly lower density and the lesion shows peripheral enhancement of centripetal fill-in on arterial phase

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Fig. 3
figure 3

Expansion of the left hepatic vein close to the lesion

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Contrast-enhanced computed tomography (CT) showed that the lesion enhanced irregularly in the left lobe of the liver. (Fig. 4)It was irregularly hypodense on plain scan with peripheral enhancement in the arterial phase and centripetal fill-in in the portal venous phase. In the delayed phases, the density of the leision was slightly higher than that of the liver parenchyma. The patient received diuretic therapy, fluid restriction, low-flow oxygen, and infection control for the management of the heart failure. After treatment his breathing difficulty improved. Then the patient was transferred to Beijing Children’s Hospital for surgery and the adhesion between the tumor and the intestine was found during the operation. The patient eventually died due to postoperative complications and multiple organ failure.

Fig. 4
figure 4

View of the patient’s moderate to severe pulmonary arterial pressure

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Discussion and conclusions

IHH is an endothelial cell neoplasm, a benign tumor, which is usually clinically silent and slowly progressive during childhood. Although almost all are asymptomatic, a small subset can produce high-output cardiac failure and cause considerable mortality. Its pathologic changes are similar to that of hepatic artery to hepatic vein or hepatic artery to portal vein arteriovenous fistula. The prognosis of the disease is poor when complications present and the mortality rate can be as high as 90% [2, 3]. We reviewed a series of recent studies of IHH-associated high-output cardiac failure between 1974 and 2018, and summarized the treatment outcomes.

Heart failure was obviously a clinical relevant complication in the 25 cases which were presented in Table 1 [2, 4,5,6,7,8,9,10,11,12,13,14,15,16,17,18,19,20,21,22]. The age of diagnosis varied ranging from 1 day to 3.5 years. Eight patients (28%) presented with pulmonary hypertension including two mild, one moderate, three severe, and two unknown. Two of them were also associated with other congenital cardiac malformations which were illustrated in detail in Table 2 [7, 11, 13, 22,23,24]. Cardiac function improved after treatment in 15 patients, achieving normal value in 2. Consequently the outcomes of IHH-associated high-output cardiac failure proved to be quite satisfying. Most of the patients discharged or achieved remarkable improvement through appropriate treatment, while only 4 of them failed to survive.

Table 1 Summary of the literature on patients presenting with IHH associated with congestive heart failure
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Table 2 Summary of patients presenting with IHH associated with pulmonary artery hypertension
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IHH can be classified as focal, multifocal, or diffuse [9]. The diagnosis of IHH relies on ultrasonography (US), CT, and magnetic resonance imaging (MRI). CT and MRI can reveal discrete lesions in patients. Diffuse lesions require baseline determination of size, cardiac and thyroid function, and coagulation profile. When IHH is associated with heart disease, cardiac structure and function can be observed by echocardiography, which can identify intra- or extracardiac disease [3]. There were 13 (52%) patients in our series that were diagnosed by echocardiography. In our case, the patient had a focal lesion, which is diagnosed primarily by ultrasonography and CT.

The pathological mechanism of CHF in IHH is associated with arteriovenous shunts in hemangiomas. The arteriovenous shunts result in a decrease of systemic blood volume as well as increase of pulmonary blood volume, thus leading to the cardiac output increase. Furthermore, aggravated by the pulmonary hypertention it finally leads to high-output CHF [21]. In fetal stage, high pulmonary vascular resistance and pulmonary pressure help maintain the fetal circulation, however, after birth the high pulmonary pressure will descend gradually within 3 months while systemic pressure of neonates will ascend with closure of oral foramen. Nevertheless, the existence of IHH can increase the load of right heart system and affect circulation transition from fetus to neonate, which further increase pulmonary vascular resistance and cause pulmonary hypertention.

Various therapies have been reported to treat IHH, including drugs, embolization, ligation, and resection [25]. (1) Drugs: steroid therapy functioned well in improving hemodynamics, reducing hepatic vascularity as well as deferring early emergency delivery in congestive heart failure fetus as recorded in literatures. There are no significant differences between single or combined drug use in the literature. (2) Embolization: embolization has been strongly suggested for provisional stabilization of fatal congestive heart failure combined with pharmacological therapy. (3) Ligation: ligation can reduce oxygen supply to hepatocytes and improve liver function. (4) Resection: surgery should be considered when medical management failed [3].Treatment methods and their outcomes as described in the literature are shown in Table 3. Among 25 patients, four ended in treatment failure (18%).

Table 3 Demographics for treatment outcomes
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Early age of onset is typical of IHH with heart failure. When IHH is detected by US, echocardiography should also be performed timely for more detailed information about cardiac structure and function. For infants in life-threatening and complicated conditions, US and echocardiography should be performed as early as possible to evaluate IHH associated with congestive heart failure and to facilitate treatment therapies. With regard to the treatment in our review, outcomes of IHH with heart failure are considered to be good.