1. Investigate her enlarged right ventricle in her heart, and if not serious enough to require intervention,
2. Perform a coil closure of her PDA
If step one uncovered a condition that would require open heart surgery, they would terminate the entire procedure without closing off the PDA and refer us to a pediatric cardiac surgeon.
Well that is exactly what happened. After about two hours of a procedure expected to last three hours, the Dr. who performs catheter procedures informed us that he did not close the PDA and proceeded to explain, with the aid of some images he printed out of Maya's innards on x-ray, that she had a rare condition called Partial Anomalous Pulmonary Venous Return of the Upper Left Lobe (PAPVR). The link provided explains the basics, at the bottom of this entry are more detailed links and quotes. From how I understand the basics, knowing very little about the complex workings of the cardiovascular system, it is a vein that grew from her left lungs' upper lobe and attached to the right atrium, creating a loop in which oxygenated blood is returned to the lungs rather than circulating throughout her body. This creates additional work for the heart, resulting in enlargement of the Right Ventricle. This enlargement creates additional pressures which result in "strengthening" of the arteries serving the heart, which really means narrowing my muscle tissue rather than the hardening of the arteries caused by cholesterol and Coronary Artery Disease seen in adults, but the consequences are the same - Pulmonary Hypertension which just gets worse. The timeline could be permanent damage by age 80 or by age 6, nobody can predict, so the correct thing to do is the operate on the heart and move the vein to the correct area of the heart (I'm still to unsure of where it will go and how they do it) via open heart surgery.
While undergoing any open-heart procedure, the surgeon will look for and repair any other repairable defects, and it is likely that Maya also has an ASD (Atrial Septal Defect) or basically a hole in the chambers of her heart as well which they saw on ultrasound wile she was sedated today. They are not sure if they actually saw one or not, because ultrasound at the extreme angle they needed to use showed a hole, but it could have been an anomaly due to the angle of view. Doppler ultrasound did not show blood flowing across the possible ASD, but the cardiologist told us this could be because a flow from the other chamber offset the pressure differential so that it looked balanced at that time) no flow across the possible "hole." In any case, an ASD, whether seen thus far or not, is likely in a PAPVR patient and will be easily and quickly sewn shut or patched during open-heart surgery.
The Surgical team of CCMC (Connecticut Children's Medical Center) will be discussing Maya's case tomorrow and we should hear back from their surgeon by later this week. We will then go back to CHOP (Children's Hospial of Philadelphia) for a second opinion. We are leaning towards CHOP as where we would like this operation performed due to their understanding of Maya's former CCAM diagnosis and removal, and the fact that they were so diligent as to first discover her heart issues while she was in for her CCAM removal surgery. They have volumes of data on her and are meticulous in their research and follow-up, and that is very important to us. Also they perform more than 1,000 pediatric open heart operations per year, so their experience is nearly unmatched.
How rare is this specific condition? I give you the following quote from: http://www.ihop-net.org/UniPub/iHOP/pm/6741000.html?pmid=2225404
"Partial anomalous pulmonary venous return.
Van Meter C, LeBlanc JG, Culpepper WS, Ochsner JL
Department of Surgery, Ochsner Clinic, New Orleans, LA 70121.
Anomalous pulmonary venous return from the left lung is an extremely rare condition that is reported sporadically and in general in case reports. From 1964 through 1988, we identified 13 patients with this condition, all of whom underwent surgical correction. This represents the single largest reported institutional experience with this anomaly. The patients ranged in age from 15 months to 40 years. Seven were asymptomatic, and six had symptoms ranging from recurrent pulmonary infection to moderate congestive heart failure. Six had anomalous venous return from the entire left lung, and seven had anomalous return from the upper lobe only. Eight of the patients had associated cardiovascular anomalies. Four of the patients underwent surgical correction via a sternotomy approach with cardiopulmonary bypass to allow correction of coexisting intracardiac anomalies. The remaining patients underwent surgical repair through a left thoracotomy. The technique included high ligation and division of a persistent left superior vena cava with anastomosis to the left atrium at the site of partial excision of the atrial appendage. There were no deaths and only one complication in our series."
So did you catch the number as of 1990? That's right, in bold there - 13, with the youngest being 15 months of age. Maya is not quite 14 months, so another record may be smashed by our daughter who exists to push the envelope of what is "typical."
I think what took the docs this long to figure out the severity of her heart problems is the fact that she is not, "failing to thrive" as is expected with compromised heart/lung function, meaning that most kids with these issues are born tiny, eat little and grow at the bottom of the curve for their age. Maya is well off of the high end of the charts for her growth, and actually was referred by our pediatrician to an endocrinologist for her size and precocious maturation rate, so go figure. If she didn't have these heart/lung issues, she might well be Babyzilla and bring on the end of western civilization by rampaging through cities, Godzilla-style, always looking for that perfect binky or bottle of milk in 55-gallon bottles. So big-baby = discount parental complaints of breathing issues and minimize significance of abnormalities until evidence is unmistakable,
*Side note to parents - if you know something is wrong, and the doctors are not finding anything or investigating all avenues, keep pushing! Your gut feelings are valid and will be vindicated. Or possibly you are delusional and need psychiatric intervention, no matter - keep pushing and eventually help will come.
So for now I leave you with the following stuff I found:
1. Bad site design with some dead links, but excellent information on the heart on its diseases in easy to understand terms:
http://www.geocities.com/HotSprings/1652/
Info from Charlotte Children's Hospital:
"Partial Anomalous Pulmonary Venous Return (PAPVR) - Summary
The four pulmonary veins return oxygen-rich blood from the lungs to the left atrium. From there, blood flows through the left ventricle to the aorta, where it is pumped to the rest of the body.
In Partial Anomalous Pulmonary Venous Return (PAPVR), one or two of the pulmonary veins return blood to the right atrium instead of the left atrium. This causes oxygen-rich blood to flow back to the lungs instead of on to the rest of the body.
Many adults with PAPVR also have an atrial septal defect (ASD).
Concerns and Symptoms
Because some oxygen-rich blood is continually flowing between the lungs and the right atrium, the right chambers of the heart may become dilated. Over time, this may cause an abnormal heart rhythm. In addition, too much blood flow to the lungs may increase the pressure in the lungs blood vessels, leading to a condition called pulmonary hypertension.
If only one of the pulmonary veins is affected by the disorder, you may not have any symptoms. If two of the veins are affected, you may experience shortness of breath during heavy exercise.
Treatment
The operation to repair PAPVR is performed under general anesthesia.
First, the surgeon makes a vertical incision in the front of the chest, opens the breastbone, and exposes the heart. Blood from the heart is redirected to a bypass machine. The bypass machine does the job of the heart and lungs during the operation.
The surgeon then opens the heart. If you have an ASD, the surgeon will create a patch that closes the defect and also redirects blood from the anomalous pulmonary vein to the left atrium.
If you do not have an ASD, the surgeon simply redirects blood from the anomalous pulmonary vein to the left atrium.
After the defects are repaired, the surgeon shuts down the heart-lung bypass machine and the heart starts beating again. The surgeon then closes the breastbone and chest incision, and applies bandages to the incision site.
Recovery
After surgery, you will be moved to the Intensive Care Unit (ICU). There they will monitor your heart function and other vital signs.
Most patients remain in ICU for at least one night after surgery. Once you are stable enough to leave the ICU, you will be moved to a regular hospital room.
You will remain in the hospital for several days after surgery. The exact length of stay depends on the type of surgery and how well you are recovering.
Before being discharged, the staff will explain any medications to take. These may include antibiotics to prevent infection while the heart heals, prior to dental work or any other procedure. They will instruct you on any activity limitations or required follow-up care.
Background
Partial anomalous pulmonary venous connection (PAPVC) with intact atrial septum is a rare congenital cardiac defect. As the name suggests, in PAPVC, a few of the pulmonary veins return to the right atrium instead of the left atrium. Thus, some of the pulmonary venous flow enters the systemic venous circulation.
Embryologically, PAPVC is similar to total anomalous pulmonary venous connection (TAPVC); however, these conditions differ in that all pulmonary venous vessels connect to the right side of the heart in TAPVC.
Anatomically, PAPVC can involve a wide variety of connections, and PAPVC from the right lung is twice as common as PAPVC from the left lung. The most common form of PAPVC is one in which a right upper pulmonary vein connects to the right atrium or the superior vena cava (SVC). This form is almost always associated with a sinus venosus type of atrial septal defect (ASD).
The right pulmonary veins can also drain into the inferior vena cava. The left pulmonary veins can drain into the innominate vein, the coronary sinus, and, rarely, the cavae, right atrium, or left subclavian vein.
Pathophysiology
A number of factors determine the ratio of pulmonary blood flow (Qp) to systemic flow (Qs). The shunt magnitude, expressed as the Qp/Qs ratio, and other factors determine development of symptoms and complications.
The most important factor is the number of pulmonary veins that drain into the systemic circulation. The more veins that anomalously drain, the more blood returns to the right side of the heart. Some authors have suggested that this defect becomes clinically significant when 50% or more of the pulmonary veins anomalously return.
In addition, the source of the returning blood plays a role in determining the clinical effect of the defect. In an individual who is upright, blood flow to the lungs is primarily directed to the lower and middle lobes. Therefore, more blood returns to the systemic venous circulation in individuals in whom the anomalous connection drains into either the right middle and lower lobes or the left lower lobe of the lung.
An associated cardiac defect, such as an ASD, may either exacerbate or alleviate disease progression.
An associated noncardiac condition (eg, scimitar syndrome) may influence disease development. Scimitar syndrome, or pulmonary venolobar syndrome, is characterized by abnormal right-sided pulmonary venous drainage in the inferior vena cava and malformation and/or hypoplasia of the right lung, with abnormal arterial supply of the right lung.
Over many years, excessive pulmonary venous return to the right side of the heart causes right atrial and ventricular dilation. This has a number of consequences, including risk of arrhythmia development, right-sided heart failure, and, rarely, development of pulmonary hypertension.
Frequency
United States
Most data regarding prevalence of this condition have been garnered from autopsy series that estimate an incidence of 0.4-0.7%. However, autopsy series may overestimate the clinical significance of this condition, as many of these cases were asymptomatic; thus, the true incidence of patients who present antemortem with this condition is lower. Clinical diagnosis of isolated PAPVC is quite rare. PAPVC occurs in approximately 10% of patients with a proven ASD.
Mortality/Morbidity
Few data are available regarding mortality due to this lesion because mortality credited to the defect occurs only in adults and the diagnosis has historically been made at autopsy. Major morbidity, including arrhythmias, right-sided cardiac failure, and, rarely, pulmonary vascular disease, also primarily occurs in adults.
Race
No data exist regarding racial predilection.
Sex
No data are available regarding sexual predilection.
Age
PAPVC is a congenital defect. Clinical evidence of this congenital defect may not be apparent until the patient reaches middle age.
CLINICAL
Section 3 of 9
Authors and Editors
Introduction
Clinical
Differentials
Workup
Treatment
Follow-up
Miscellaneous
References
History
Children with partial anomalous pulmonary venous connection (PAPVC) usually remain asymptomatic and are referred based on an incidentally noted cardiac murmur. Symptoms may occur in older patients and may be secondary to right-sided volume overload or pulmonary vascular obstructive disease.
Determining the natural history of this condition was difficult before the era of direct cardiac imaging (ie, echocardiography, cardiac catheterization), as the diagnosis was made only postmortem.
The development of complications from PAPVC clearly depends on how many pulmonary veins abnormally return to the right heart. A single anomalous vein is usually not hemodynamically significant and, hence, does not produce any symptoms.
About 10% of patients with an ASD also have PAPVC and may have symptoms of right-sided overload.
Dyspnea may occur in adults but is rare in children. A child may experience exercise intolerance as a symptom in cases in which more than 50% of pulmonary veins anomalously drain.
Palpitations may reflect cardiac arrhythmias, which are almost always supraventricular in origin, but this is rare in childhood.
Hemoptysis is a rare symptom that reflects either chest infection or the development of pulmonary vascular disease.
Chest pain may be evidence of right heart ischemia but does not occur in childhood. More commonly, chest pain may be a manifestation of recurrent bronchitis.
Associated defects (either cardiac or extracardiac) can produce symptoms.
Peripheral edema can occur in adults with cardiac failure.
Physical
Physical examination findings are usually more revealing than the history, but positive findings depend on the volume of abnormal pulmonary venous return to the right heart. If only a single vein is anomalous, the physical examination findings may be normal. In a patient with a larger volume of abnormal veins, physical examination findings are similar to those of an uncomplicated ASD. These findings include the following:
Left parasternal lift reflects right ventricular dilation. Impulse in the second left intercostal space reflects pulmonary artery dilation.
A soft systolic ejection murmur is heard over the pulmonary area, reflecting turbulence in the pulmonary trunk due to increased right ventricular ejection volume. The second heart sound is always widely split but may have normal respiratory variation.
In healthy individuals, inspiration increases systemic venous return to the right heart, causing a delay in the pulmonic closure component of the second sound. This phenomenon also occurs in patients with PAPVC who have an intact atrial septum. However, in patients with PAPVC and ASD, volume flow into the right heart is always increased, and respiration further augments that flow. Therefore, splitting of S2 proceeds from wide during expiration to wider during inspiration. This does not occur in patients with a significant ASD, in whom second heart spitting is wide and fixed. In the presence of an ASD, variations in systemic venous return during respiration are counterbalanced by reciprocal changes in flow through the ASD, maintaining total right ventricular flow more or less constant. A mid-diastolic murmur due to increased transtricuspid right ventricular filling may be heard over the tricuspid valve area at the lower left sternal border.
Cyanosis does not occur, even in older patients in whom pulmonary hypertension develops, as a right-to-left shunt cannot develop in the absence of an atrial septal communication.
Right-sided heart failure signs in adults include hepatomegaly, jugular venous distension, ascites, and peripheral edema.
Pulmonary vascular disease may occur in older adults, although this is rare. Clinical signs of pulmonary hypertension include a right ventricular parasternal lift, absence of systolic murmur, narrowly split S2 with a loud pulmonic component, and, occasionally, an early, high-frequency murmur of pulmonic regurgitation. Cyanosis does not occur in the presence of an intact atrial septum.
Causes
No causes of this condition are known. No evidence has implicated common teratogens (eg, drugs, infections) in the genesis of PAPVC. No evidence for a genetic predisposition has been reported."
http://www.charlottechildrenhospital.net/body.cfm?id=404&oTopID=404
Do these pictures look like a child failing to thrive to you?
