Holmes Heart-A Simple Antenatal Diagnosis of a Complex Cardiac Anomaly? Fetal Echocardiographic Findings and Review

Weichert, J.; Axt-Fliedner, R; Gembruch, Ulrich; Hartge, D.R.

doi:10.1111/j.1747-0803.2011.00621.x

Cited by 6 publications

(4 citation statements)

References 12 publications

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“…A small series of eight cases of DILV described L-MGA in all cases [10]. The frequency of a Holmes heart is given in the literature as 15 % [11]. In our collective, this variant was seen in 16.6 %.…”

Section: Discussionsupporting

confidence: 44%

Prenatal Diagnosis, Associated Findings, and Postnatal Outcome in Fetuses with Double Inlet Ventricle (DIV)

et al. 2022

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Purpose To assess the spectrum of associated cardiac anomalies, the intrauterine course, and postnatal outcome of fetuses with double inlet ventricle (DIV). Methods Retrospective analysis of prenatal ultrasound of 35 patients with DIV diagnosed between 2003 and 2021 in two tertiary referral centers in Germany. All fetuses underwent fetal echocardiography and a detailed anomaly scan. Postnatal outcome and follow-up data were retrieved from pediatric reports. Results 33 cases of DIV were correctly diagnosed prenatally. 24 fetuses (72.7%) had a double inlet ventricle with dominant left (DILV), 7 (21.2%) with dominant right ventricular morphology (DIRV), and 2 cases (6%) with indeterminate morphology (DIIV). 4 (16.6%) were Holmes hearts. 5 of the 7 fetuses (71.4%) with DIRV had a double outlet right ventricle (DORV). Malposition of the great arteries was present in 84.8%. Chromosomal abnormalities were absent. Termination of pregnancy was performed in 8 cases (24.2%). 24 fetuses (72.7%) were live-born. 5 (20.8%) were female and 19 (79.2%) were male. The median gestational age at birth was 38+2.5 weeks. All but one child received univentricular palliation. The median follow-up time was 5.83 years with an adjusted survival rate of 91.6% (22 of 24 live-born children). There was one case of Fontan failure at 15.7 years. Conclusion DIV remains a major cardiac malformation although both prenatal diagnostics and cardiac surgery have improved over the years. The course of pregnancy is commonly uneventful. All children need univentricular palliation. The children are slightly physically limited, develop a normal intellect, and attend school regularly.

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Section: Discussionsupporting

confidence: 44%

Prenatal Diagnosis, Associated Findings, and Postnatal Outcome in Fetuses with Double Inlet Ventricle (DIV)

et al. 2022

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“…Holmes heart is a rare type of single‐ventricle cardiac malformation, accounting for fewer than 1% of congenital heart defects . An absent right ventricular sinus prevents blood from entering the right ventricle, resulting in a double inlet left ventricle.…”

Section: Discussionmentioning

confidence: 99%

Holmes heart and tetralogy of Fallot in association with PHACE

Peterman

Morley

Yan

et al. 2017

Pediatric Dermatology

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PHACE is an association between large infantile hemangiomas and brain, arterial, cardiac, and/or ocular abnormalities. Aortic or subclavian aberrations are the most common cardiovascular anomalies in PHACE, whereas complex congenital heart disease is rare. We report a case of Holmes heart and three cases of tetralogy of Fallot in PHACE association. with complex congenital heart disease, but we excluded 1 previously reported with tricuspid atresia. 3 We report a case of Holmes heart and three cases of tetralogy of Fallot (ToF) with PHACE.A full-term infant girl was diagnosed prenatally with single-ventricle heart disease. At birth, she had a holosystolic murmur at the left sternal border and an upper systolic ejection murmur consistent with pulmonary stenosis. She also had a 4-9 7-cm telangiectatic patch within an area of pallor on the left cheek and ear, consistent with a reticular infantile hemangioma (IH) (Figure 1).Echocardiography revealed a double-inlet left ventricle with absent right ventricular sinus, normally related great arteries, and an infundibular outflow chamber leading to a unicommissural pulmonary valve with severe pulmonary stenosis indicating Holmes heart. She also had a mild distal aortic arch obstruction. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of the brain and neck were normal, as was ophthalmologic examination.At age 4 months, she underwent a bidirectional Glenn procedure with division of the main pulmonary artery, coarctation resection, and atrial septectomy, followed by Fontan completion at age 2 years.Her hemangioma did not proliferate, so medical therapy was not initiated. She underwent eight pulsed dye laser treatments, with marked improvement.F I G U R E 1 Telangiectatic patch within an area of pallor involving the left temple, cheek, ear, and postauricular area (Patient 1)

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“…In the vast majority of cases, there is ventriculoarterial discordance with a left anteriorly located ascending aorta from the RV and pulmonary trunk arising right posteriorly from the dominant LV (74%) 75 . Concordant ventriculoarterial connections with the aorta arising off the dominant morphologic left ventricle, whereas cases in which the pulmonary artery arises off the anterior hypoplastic right outlet chamber are considerably rare (15%; referred to as Holmes’ heart) 76 . According to van Praagh et al, the exact characterization of the morphology of the ventricular myocardium constitutes the most important aspect for proper identification of the underlying anatomic variant 72 .…”

Section: Double‐inlet Left Ventriclementioning

confidence: 99%

“…75 Concordant ventriculoarterial connections with the aorta arising off the dominant morphologic left ventricle, whereas cases in which the pulmonary artery arises off the anterior hypoplastic right outlet chamber are considerably rare (15%; referred to as Holmes' heart). 76 According to van Praagh et al, the exact characterization of the morphology of the ventricular myocardium constitutes the most important aspect for proper identification of the underlying anatomic variant. 72 Semilunar valve stenosis or atresia (40%) and aortic arch abnormalities, such as coarctation and interrupted arch (12%) and AV valve abnormalities (39%), 75,77 may be associated with a single LV.…”

Section: Doub Le-inle T Lef T Ventri Clementioning

confidence: 99%

A “holistic” sonographic view on congenital heart disease: How automatic reconstruction using fetal intelligent navigation echocardiography eases unveiling of abnormal cardiac anatomy part II—Left heart anomalies

Weichert

2021

Echocardiography

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Volume ultrasound has been shown to provide valid complementary information on fetal anatomy. Four‐dimensional assessment (4D) of the fetal cardiovascular system using spatial–temporal image correlation (STIC) allows for detailed examination of a highly complex organ from the early second trimester onward. There is compelling evidence that this technique harbors quite a number of diagnostic opportunities, but manual navigation through STIC volume datasets is highly operator dependent. In fact, STIC is not incorporated yet into daily practice. Application of the novel fetal intelligent navigation echocardiography (FINE) considerably simplifies fetal cardiac volumetric examinations. This automatic technique applied on cardiac volume datasets reportedly has both high sensitivity and specificity for the detection of congenital heart defects (CHDs). Part I reviewed current data regarding detection rates of CHDs and illustrated the additional value of an automatic approach in delineating cardiac anatomy exemplified by congenital lesions of the right heart. In part II of this pictorial essay, we focused on left heart anomalies and aimed to tabulate recent findings on the quantification of normal and abnormal cardiac anatomy.

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Holmes Heart-A Simple Antenatal Diagnosis of a Complex Cardiac Anomaly? Fetal Echocardiographic Findings and Review

Cited by 6 publications

References 12 publications

Prenatal Diagnosis, Associated Findings, and Postnatal Outcome in Fetuses with Double Inlet Ventricle (DIV)

Prenatal Diagnosis, Associated Findings, and Postnatal Outcome in Fetuses with Double Inlet Ventricle (DIV)

Holmes heart and tetralogy of Fallot in association with PHACE

A “holistic” sonographic view on congenital heart disease: How automatic reconstruction using fetal intelligent navigation echocardiography eases unveiling of abnormal cardiac anatomy part II—Left heart anomalies

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