High Output Heart Failure

Eva Ilse Rubio, MD

DIFFERENTIAL DIAGNOSIS

Common

Intracardiac Shunt
Vein of Galen Aneurysmal Malformation
Anemias

Less Common

Vascular Malformations
Hemangioendothelioma
Teratoma

Rare but Important

Parkes-Weber
Chorioangioma

ESSENTIAL INFORMATION

Key Differential Diagnosis Issues

Age of patient: Newborn, toddler, child, or adolescent?
Prior medical history
- Underlying congenital heart defect or aortic anomaly
- Infectious history or postinfectious syndrome
- Chronic medical conditions or therapy
Intracardiac vs. extracardiac cause?

Helpful Clues for Common Diagnoses

Intracardiac Shunt
- Ventricular septal defect
  - Most common cardiac anomaly in general population
  - Radiograph findings: Hyperexpanded lungs, large round heart, plump pulmonary vessels most conspicuous at hila
- Atrioventricular septal defect
  - Commonly associated with Down syndrome
  - Radiograph findings: Hyperexpanded lungs, large round heart, plump pulmonary vessels most conspicuous at hila
- Anomalous coronary artery
  - Most commonly, left coronary artery arises from pulmonary artery origin
  - Preferential flow away from myocardial muscular bed into pulmonary circulation results in ventricular ischemia
  - Imaging features: Marked cardiomegaly (CM); left ventricular and atrial enlargement conspicuous on lateral view
Vein of Galen Aneurysmal Malformation
- Congenital arteriovenous fistulous communicate between midline intracranial arteries and vein of Galen or other fetal venous structures
- Poor prognosis
  - Choroidal type
  - Fetal hydropic changes
  - Congestive heart failure with intracranial bruit
- Intracranial findings
  - Large midline vascular anomaly
  - Hydrocephalus
  - Encephalomalacia
Anemias
- Sickle cell disease
  - Longstanding anemia leads to global CM
  - Physiologic contributions: High output of anemia, poor oxygen delivery to coronary arteries, pulmonary arterial hypertension
  - More common in older children, adolescents
- β-thalassemia
  - Severe anemia leads to global CM
  - Physiologic contributions: High output anemia, elevated iron levels/deposition from transfusions
  - May manifest earlier in childhood

Helpful Clues for Less Common Diagnoses

Vascular Malformations
- Typical locations: Head, neck, extremities, liver
- Classification of vascular anomalies (these lesions grow commensurate with child)
  - High-flow vascular lesions (arteriovenous malformations and arteriovenous fistulae)
  - Low-flow vascular lesions (venous, lymphatic, venolymphatic)
Hemangioendothelioma
- a.k.a. infantile hepatic hemangioma
  - Hypervascular liver mass seen in infants
  - May have cutaneous hemangiomas, especially multiple
- Imaging
  - May be multiple rounded lesions or single dominant lesion
  - US: Hypoechoic
  - CT: Round, enhancing lesions; may enhance from periphery to center
  - MR: Bright on T2, isointense/dark on T1
- Cardiovascular sequelae/manifestations
  - Significant arteriovenous shunting may lead to high output failure
  - Aorta may be diminutive distal to lesion
  - Disseminated intravascular coagulopathy may occur
Teratoma
- Germ cell tumor presumably arising from multipotential cells
- Typical locations
  - Sacrococcygeal region, arising from Hensen node
  - Neck
  - Oropharynx
  - Abdomen
  - Retroperitoneum
- More common in females
- Findings associated with increased risk for congestive heart failure
  - Larger lesions
  - Significant solid tissue component
  - Large feeding vessels/robust internal vascularity
  - Intralesional hemorrhage
  - Placentomegaly
  - Hydropic changes in fetus
- Sacrococcygeal teratoma (SCGT)
  - Classified into types 1-4 based on extrapelvic vs. intrapelvic components
  - SCGT findings associated with heart failure: Aortic velocity > 60 cm/sec; IVC diameter > 4.1 mm (21-28 weeks gestation); reversed hypogastric flow
- Cervical teratomas
  - Differential consideration is lymphatic malformation if multicystic
  - Other considerations: Airway management, intracranial involvement

Helpful Clues for Rare Diagnoses

Parkes-Weber
- Rare extremity soft tissue overgrowth syndrome due to vascular anomaly
  - Limb overgrowth
- Combined vascular malformation; capillary malformation, high-flow AVMs ± lymphatic malformation
- May result in high output heart failure
Chorioangioma
- Benign, rare placental tumor
- Consists of small caliber vascular structures and stroma
- Often seen at base of umbilical cord, with vascular supply arising near or from umbilical vessels
- Potential fetal sequelae
  - Hydropic changes
  - Growth restriction

Image Gallery

AP radiograph in this 8-month-old child who presented with wheezing and grunting shows new and significant enlargement of the cardiac silhouette

. An anomalous coronary artery was discovered.

Lateral radiograph of the same child demonstrates left atrial and ventricular enlargement with the posterior border of the heart

overlapping with the spine and causing airway compression

(Left) AP radiograph shows mild cardiac enlargement

and increased caliber of the pulmonary vessels

in a child with a ventricular septal defect. (Right) AP radiograph shows marked cardiomegaly

and florid pulmonary vascular congestion

in a 3-month-old child with diabetic embryopathy and an unbalanced atrioventricular septal defect.

(Left) AP radiograph shows a patient with cardiomegaly

due to severe complex congenital heart disease, including situs inversus, transposition of the great vessels, and large atrial/ventricular septal defects. (Right) Axial T2WI MR of the same patient shows global enlargement of all 4 chambers of the heart