Pectus Excavatum, Patent Foramen Ovale, and Migraine in the Same Patient

Abstract

Background

A patent foramen ovale (PFO) can be found in patients with pectus excavatum (PE); PFO can be associated with migraines.

Case summary

A 22-year-old woman presents a PE and a history of recurrent migraines. Pulsed oxygen saturation at effort decreased from 97% to 92%. A transthoracic echocardiography showed a PFO. Five years after the Ravitch procedure, pulsed oxygen saturation remained stable at effort, no PFO was shown on transthoracic echocardiography, and migraine disappeared.

Discussion

Our observation is the first description of the PE, PFO, and migraine association. The correction of PE has allowed the closure of the PFO and improved migraines.

Take-Home Messages

This case report suggests that a patient presenting a PE should undergo a comprehensive cardiorespiratory evaluation. Similarly, patients with a symptomatic PFO and PE should benefit from a multidisciplinary discussion involving thoracic surgeons to determine the best approach to treat the PFO.

Graphical Abstract

Pectus excavatum (PE) is the most frequent deformation of the anterior thoracic wall. This thoracic deformation is generally considered benign; thus, many authors suggest that surgical correction in young adults is purely for esthetic and social reasons. Other studies support improved cardiac function and aerobic capabilities after surgery, explained by the decompression of the heart's chambers.

Take-Home Messages

• •This case report suggests that a patient presenting a pectus excavatum should undergo a comprehensive cardiorespiratory evaluation.
• •Similarly, patients with a symptomatic patent foramen ovale and pectus excavatum should benefit from a multidisciplinary discussion involving thoracic surgeons to determine the best approach to treat the patent foramen ovale.

We report a case of patent foramen ovale (PFO) closure after surgical correction of PE, accompanied by an improvement of gas exchange at maximal effort and significant improvement in migraine episodes with aura.

History of Presentation

A 22-year-old woman was referred for correction of a severe PE (Haller index: 5.3, correction index: 48%; Figure 1A).

Figure 1.

Thoracic CT Scan

(A) Preoperative thoracic CT scan (Haller index: 5.3, correction index: 48%). (B) Postoperative thoracic CT scan (1 year after surgery) (Haller index: 2.65, correction index: 14%). CT = computed tomography.

Past Medical History

Her only medical history consisted of migraines with aura (approximately once a month), prophylactically treated with amitriptyline since the age of 13 years.

Investigations

Initial transthoracic echocardiography (TTE) did not show any valve disease. Right and left ventricular functions were normal despite compression of the right atrium exerted by the thoracic wall deformity. An incremental exercise test on a cycle ergometer with progressive stages of 15 W was performed. A peak aerobic capacity of 25.3 mL/kg/min (representing 72% of the theoretical maximum) was attained. Pulsed oxygen saturation (SpO₂) went from 97% to 92% at the end of the effort. A TTE with a microbubble test and Valsalva maneuver was then performed, showing a moderate right-to-left shunt (RLS) through a PFO during the effort (Figure 2A).

Figure 2.

Transthoracic Echocardiography With a Microbubbles Test and Valsalva

(A) Preoperative TEE: the passage of microbubbles (arrow) confirms the presence of a right-to-left shunt during the effort (moderate PFO); (B) Postoperative TEE (2 years after surgery): The absence of passage of microbubbles confirms the absence of a right-to-left shunt during the effort. PFO = patent foramen ovale; TEE = transthoracic echocardiography.

Management (Intervention)

The patient underwent a modified Ravitch-type surgical repair of her PE with the placement of a retrosternal metal bar.¹ The postoperative course was uneventful. The patient resumed her work and daily activities 2 months later.

Outcome and Follow-Up

The retrosternal metal bar was removed under local anesthesia 14 months after surgery with excellent esthetic and radiological results (Figure 1B).

The patient underwent a new exercise test 2 years after the surgery, under the same conditions as the previous test. Aerobic capacity, respiratory pattern, and pulsed O₂ were similar to preoperative values. However, SpO₂ remained stable during the test, varying from 97% to 98%. Follow-up TTE with microbubble test and Valsalva maneuver was normal with no evidence of RLS (Figure 2B). Five years after her surgery, she reported only one episode of migraine with aura.

Discussion

Pectus excavatum

PE is a chest wall deformity that causes a depression of the anterior chest wall. PE is often asymptomatic, but other musculoskeletal conditions, such as scoliosis and connective tissue disorders (eg, Marfan syndrome, Ehlers-Danlos syndrome), may be associated with it. Rarely PE could be associated with complex clinical scenarios, including myocardial ischemia, bronchomalacia, syncope related to compression of the inferior vena cava or right ventricle, congestive hepatopathy, and arrhythmia.

PE is often linked to intolerance to exertion through various mechanisms, including limiting expansion of current volume, gas-exchange abnormalities due to an enlarged alveoli-capillary gradient in O₂ related to a PFO, and finally, cardiovascular issues.²

Therapy includes conservative strategies such as external negative pressure (Vacuum Bell device) or surgical intervention. Insertion of silicone implants, a minimally invasive technique (Nuss procedure), or open chondro-sternal correction (Ravitch procedure) has largely been documented in the literature.³

Although a functional benefit after PE correction is controversial and difficult to document especially at rest, recent studies show that cardiac function may improve after surgical correction.⁴

PE and patent foramen ovale

PFO is due to postnatal adhesion failure in primary and secondary atrial septum and may be responsible for transient episodes of right-to-left shunting; indeed, the normally positive left-to-right pressure gradient may be reversed. This abnormality is primarily identified during exercise or Valsalva maneuver (attempted exhalation against a closed glottis).⁵

The prevalence of PFO at echocardiography in the general population is approximately 20%.⁵ Often asymptomatic, PFO can be associated with many conditions, including decompression sickness in divers, high-altitude pulmonary edema, sleep apnea syndrome, migraines with aura, and paradoxical embolism with a particularly high risk of stroke.⁵

The standard diagnostic technique for PFO includes TTE or transesophageal echocardiography with a microbubble test; the sensitivity and specificity are both >90%.⁵

The association of PE with PFO is poorly documented, but the preoperative study of cardiac function is not systematically carried out. Only one study has reported systematic preoperative TTE.¹ The prevalence of PFO in this PE cohort was 29% (20/70), slightly higher than that in the general population. The link between PE and PFO is explained by the atrial septal distortion imposed by the thoracic deformity and by the modified geometry of the cardiac chambers, which may favor a venous return flow via the inferior vena cava directed toward a potential PFO.⁶ The validity of this hypothesis is supported by the observation that PFO closure occurs in half of the cases after correction of the PE.¹

Association between PFO and migraine

The association between PFO and migraine is widely documented in the literature, and a decrease or total disappearance of migraine episodes following percutaneous PFO closure has often been reported.⁶ It is, however, unknown if spontaneous closure of PFO after surgical correction of PE is also associated with the resolution of migraine attacks.

Robust evidence exists about the association between migraine with aura and increased incidence of cardiovascular disease, in particular, ischemic stroke. Following the closure, there may be an increase in migraine attacks for a short period of time, but afterward, many patients experience an improvement in their condition.⁷

However, published data are inconsistent, and except for the prevention of a relapse of stroke, there is currently no clear indication of PFO closure for migraine.⁸

In a recent study, patients presenting migraine headaches with aura and PFO displayed an increased prothrombotic phenotype, sustained by an altered oxidative stress status.⁹ Moreover, PFO may cause RLS, leading to neurological chronic diseases with episodic manifestations, such as migraines. According to these pathophysiological mechanisms, PFO closure could prevent RLS and reduce migraine symptoms.¹⁰ The selection of patients to be treated is the challenge for future randomized trials.

Conclusions

Our observation is original because it is the first to describe this association of PE, PFO, and migraine in the same patient.

The correction of PE allowed the indirect closure of the PFO and substantially improved migraine attacks. After more than 5 years of surgery, the clinic and tests confirm the stability of the results over time. We suggest reserving endovascular PFO treatment for cases of failed PFO closure after a PE correction, or in case of recurrent symptoms, it relates to persistent PFO. This case report suggests that a multidisciplinary approach should be used when investigating patients with PE, including respiratory and cardiac stress tests with a noninvasive microbubble test. Similarly, patients presenting with a symptomatic PFO and PE should benefit from a multidisciplinary discussion involving thoracic surgeons to determine the best approach (transcutaneous closure or surgical correction of the PE) to treat the PFO.

Funding Support and Author Disclosures

The authors have reported that they have no relationships relevant to the contents of this paper to disclose.