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Prenatal diagnosis of left ventricular aneurysm and diverticulum

Abstracts

We report two cases of localized left ventricular (LV) protrusion, an entity that has been described in the literature as aneurysm or diverticulum. Both cases had different outcomes from those previously reported. Little is known on the incidence and natural history of these rare anomalies, whose progression may be asymptomatic or lead to severe complications and death in the prenatal period. The therapeutic approach should be customized.

Prenatal care; ultrasonography prenatal; pericardial effusion


Descrevemos dois casos de protrusão localizada do ventrículo esquerdo (VE), entidade que tem sido descrita na literatura como aneurisma ou divertículo. Em ambos os casos, observou-se uma evolução distinta da anteriormente relatada. A incidência e história natural dessas raras anomalias são pouco conhecidas, podendo evoluir de forma assintomática ou gerar graves complicações e até o óbito no período pré-natal. A abordagem terapêutica deve ser individualizada.

Cuidado pré-natal; ultrassonografia pré-natal; derrame pericárdico


Describimos dos casos de protrusión localizada del ventrículo izquierdo (VI), entidad que se describe en la bibliografía como aneurisma o divertículo. En ambos casos se observó una evolución distinta a la anteriormente relatada. La incidencia e historia natural de estas raras anomalías son poco conocidas, pudiendo evolucionar de forma asintomática o generar graves complicaciones e incluso el óbito en el período prenatal. El abordaje terapéutico debe ser individualizado.

Cuidado prenatal; ecografía prenatal; derrame pericárdico


CASE REPORT

Prenatal diagnosis of left ventricular aneurysm and diverticulum

Marcia F. A. Barberato; Silvio H. Barberato; Cristiane N. Binotto; Mary Julianne M. Cavalcanti; Ana Paula Passos; Nelson I. Miyague

Unidade de Saúde Mãe Curitibana, Prefeitura de Curitiba, Curitiba, PR - Brazil

Mailing address

ABSTRACT

We report two cases of localized left ventricular (LV) protrusion, an entity that has been described in the literature as aneurysm or diverticulum. Both cases had different outcomes from those previously reported. Little is known on the incidence and natural history of these rare anomalies, whose progression may be asymptomatic or lead to severe complications and death in the prenatal period. The therapeutic approach should be customized.

Key words: Prenatal care; ultrasonography prenatal; pericardial effusion.

Case Report 1

A 34-year-old patient, gravida 2, para 1, on the 16th week of gestation, was referred to the maternal-fetal medicine department due to the finding of pericardial effusion on routine prenatal echocardiography. The fetal anatomy was otherwise normal. Fetal echocardiography showed significant pericardial effusion and a finger-like protrusion with a narrow neck located in the LV apex, measuring approximately 5 x 5.7 mm (Figure 1). No thrombi or other cardiac abnormalities were found. Pericardiocentesis was indicated and successfully performed at 20 weeks of gestation. Drainage of 6 mL of serosanguineous fluid was followed by immediate pulmonary expansion. A mild increase of the pericardial effusion was observed in post-intervention control tests in relation to post-puncture tests, however always with preserved left ventricular function and good pulmonary expansion. On the thirty-seventh week, fetal death was diagnosed on a control echocardiogram. Necropsy was not performed because of family's refusal.


Case Report 2

A 25-year-old patient, gravida 1, para 0, on the 30th week of gestation was referred for fetal echocardiography due to suspected cardiac malformation. The study showed dilated LV with decreased systolic function (fractional shortening = 25%) and a sac-like protrusion with a wide neck in the LV lateral wall below the mitral valve (Figure 2). Paradoxical systolic bulging of this structure, which measured 12 x 13 mm, was observed. Color and pulsed Doppler demonstrated flow inside the structure, thus confirming continuity with the LV. Watchful waiting during pregnancy did not show either hemodynamic decompensation or worsened ventricular function. Cesarean section was performed on the 36th week due to maternal infection (pyelonephritis), delivering a preterm infant weighing 2.5 kg with a one and five-minute Apgar score of 8 and 9, respectively. Neonatal echocardiography confirmed the malformation and showed preserved LV systolic function. Watchful waiting was chosen, and, to date, the seven-month-old infant progresses asymptomatically, not requiring any therapeutic intervention.


Discussion

Congenital protrusions of the heart ventricular walls have been described in the literature as diverticula or aneurysms. Their incidence is unknown, because they can progress asymptomatically or lead to hemodynamic complications and death in the prenatal period1. The underlying pathophysiological processes are little understood; however, it is hypothesized that they result from a localized weakening of the ventricular wall due to an interruption in the development during embryogenesis, infection, or ischemia2,3. Although there is no definitive consensus for the differentiation between these entities, some criteria have been proposed.

Diverticula are characterized by a narrow connection with the ventricle and presence of myocardial fibers in the composition of their walls, thus enabling efficient systolic contraction in some cases1. They may be isolated3 or associated with interventricular4 and defects of the mid-thoracoabdominal line2. They affect either the right3,4 or the left ventricular chamber1, more commonly in the apex1,3,4. Most cases reported in the prenatal period are from findings of tests performed during the second trimester of pregnancy, because diverticula are usually smaller structures than aneurysms and do not cause cardiovascular compression. Thus, the prognosis seems to be favorable, and most of the neonates are asymptomatic at birth5. However, depending on their location and association with other anomalies, diverticula may trigger heart failure, arrhythmia, rupture, large pericardial effusion, and hydrops fetalis1,2,5,6. In two cases with pericardial effusion diagnosed in early phases of pregnancy, the risk of pulmonary hypoplasia led to termination of pregnancy, a procedure that is legally permitted in the country where these cases were reported5,6. It is worth pointing out that, in the absence of hemodynamic impairment, pericardial effusion usually has a favorable outcome and spontaneous resolution2. Some authors have reported the benefits of pericardiocentesis in cases with pericardial effusion associated with heart failure, permitting normal lung development and a favorable outcome2,3,7. This was not observed in case 1 reported here, which was diagnosed as a diverticulum. Pericardiocentesis was performed because the pregnancy was in its early stages. Based on previous experience with good outcomes after pericardiocentesis, watchful waiting was recommended after puncture; however, fetal death occurred close to term. Since there were no signs of hemodynamic decompensation in the serial tests, the major hypothesis is of ruptured diverticulum, despite its unchanged size. Our experience, therefore, contrasts with the usually favorable outcome attributed to diverticula. Another aspect that should be pointed out is that the finding of isolated pericardial effusion with no obvious cause requires the investigation of uncommon abnormalities, such as ventricular or atrial diverticulum8.

On the other hand, aneurysms are characterized by a unilobulated or multilobulated bulging of a ventricular wall segment, with a typical wide neck, fibrotic tissue and akinesia9. They can also be asymptomatic4, but lead to a higher risk of complications such as rupture, embolism, arrhythmia, pericardial effusion, heart failure and hydrops fetalis10. Indication of surgical treatment after birth is controversial, with some authors suggesting surgical excision only in symptomatic patients, and others supporting surgery regardless of symptoms9. Case 2, diagnosed as an aneurysm, showed an association with dilatation and impaired LV systolic function in the initial moment, thus suggesting a worse outcome. Again, unlike previous reports of the literature, we observed a good outcome, with no hemodynamic decompensation during the follow-up until delivery. Additionally, the LV systolic function returned to normal and a good clinical outcome was observed after delivery. Again, the experience with this specific case is different from the usually poor outcome attributed to aneurysms. In our opinion, these peculiarities result from the fact that these two entities are distinct presentations of the same anomaly. Although differences exist in relation to the macroanatomical and histological characteristics, both represent dysplastic myocardial areas with different degrees of severity, probably acquired during early phases of embryogenesis. Additionally, the lack of consensus on the definition of terms (diverticulum versus aneurysm) and their interchangeable utilization may have led to imprecise diagnoses in the past, thus affecting the perception of the clinical outcome of these conditions. The treatment proposed in the cases reported (serial assessment, pericardiocentesis, watchful waiting) followed the few recommendations available in the literature, which are always based on the experience with small case series.

In conclusion, the diagnosis and differentiation between ventricular diverticula and aneurysms may be made in the prenatal period, from the early phases of pregnancy. Although a better prognosis has been attributed to fetuses with diverticula, adverse outcomes may result from any of the two entities. The true natural history of these rare conditions is little known and further interventions in fetuses with similar conditions should be customized.

Potential Conflict of Interest

No potential conflict of interest relevant to this article was reported.

Sources of Funding

There were no external funding sources for this study.

Study Association

This study is not associated with any post-graduation program.

References

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  • Correspondência:
    Silvio Henrique Barberato
    Rua Saint Hilaire, 122/203, Água Verde
    80.240-140, Curitiba, PR - Brasil
    E-mail:
  • Publication Dates

    • Publication in this collection
      13 Oct 2009
    • Date of issue
      Aug 2009

    History

    • Received
      12 July 2008
    • Reviewed
      07 Oct 2008
    • Accepted
      13 Oct 2008
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